University of California, San Francisco …labmed.ucsf.edu/dnld/faculty-path-sdearmond-CV.pdfPrepared: November 3, 2011 1 of 56 University of California, San Francisco CURRICULUM VITAE

Prepared: November 3, 2011

1 of 56

University of California, San Francisco

CURRICULUM VITAE

Name: Stephen J De Armond

Position: Professor, Step 7

Pathology School of Medicine

Address: Box 0803, 1855 Folsom Street, MCB, 505

University of California, San Francisco San Francisco, CA 94143

Voice: 415 502-6603 Fax: 415 502-8545 email: [email protected]

EDUCATION

1953 - 1957 Bellarmine College Preparatory, San Jose, CA

1957 - 1961 Santa Clara University, Santa Clara, CA

1961 - 1963 University of Utah, Salt Lake City, UT

B.S. Cum Laude, Biology

1963 - 1966 University of Michigan, Ann Arbor, MI

M.S. Physiology

1966 - 1972 The Medical College of Pennsylvania, Philadelphia, PA.

PhD Physiology/Biophysics

1968 - 1975 The Medical College of Pennsylvania, Philadelphia, PA

M.D.

1975 - 1976 Stanford University Medical Center

Intern Pathology


Resident Pathology


Post-doctoral scholar

Neuropathology


Fellowship Neuropathology

LICENSES, CERTIFICATION

1977 Medical licensure, California


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1981 Certified, American Board of Pathology (Anatomic Pathology and Neuropathology)

PRINCIPAL POSITIONS HELD

1980 - 1983 VA Medical Center, Palo Alto, CA Research Associate/Physician

Pathology

1983 - 1987 University of California, San Francisco

Assistant Professor

Pathology and Neurology


Associate Professor

Pathology and Neurology


Co-Director Neuropathology Unit


Director Neuropathology (Pathology)

1991 - present University of California, San Francisco

Professor Pathology and Neurology

OTHER POSITIONS HELD CONCURRENTLY

1971 - 1972 Medical College of Pennsylvania Instructor Neuroanatomy

1980 - 1981 Stanford University Medical Center Clinical Instructor Pathology

1981 - 1983 Stanford University Medical Center Clinical Assistant Professor

Pathology

1983 - present San Francisco General Hospital Consultant Neuropathology

1983 - 2000 Veterans Administration Medical Center, San Francisco

Consultant Neuropathology

1990 - present Mt. Zion Medical Center Consultant Neuropathology

HONORS AND AWARDS

1963 Research Training Fellowship, NSF


1975 Dean's Prize for Outstanding Achievement and Contribution to the Medical College of Pennsylvania


1980 Research Associate Award, Veterans Administration

1983 Academic Senate Grant, UCSF

1983 Research Evaluation and Allocation Committee


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Grant, UCSF

1984 Academic Senate Grant, UCSF

1985 John D. French Foundation Grant for Alzheimer's Disease

1986 Visiting Professor of Neuropathology, The University of Pennsylvania, Department of Neuropathology

1987 Plastic Surgery Educational Foundation, First Place-Junior Clinical Category for paper "Etiology of Cleft Lip: A Physiology Basis," in collaboration with Dr. Steve Schindel

1988 Alpha Omega Alpha Honor Medical Society: elected to membership in the Medical College of Pennsylvania Chapter

1997 Acknowledged as major contributor to Nobel Prize in Medicine for Stanley Prusiner

1998 Landmark Article, American Academy of Neurology, for my widely cited work on prion diseases (Neurology 50:1-2, 1998)

1998 17th Annual Nobuhisa Baba Memorial Lecturer, Department of Pathology, School of Medicine, Ohio State University: "Prions: Invaders from Innerspace".

1998 The Robert E. Stowell Lectureship, School of Medicine, University of California at Davis: "In recognition of Dr. DeArmond's contribution to the pioneering research that led to the novel concept of prion-based diseases".

1999 President of the American Association of Neuropathologists (AANP)

2002 The Peter Lampert Lecturer, Department of Pathology, University of California, San Diego

2003 Vice President, International Society of Neuropathology

2004 AANP Weil Award for the "Best Paper on Experimental Neuropathology, "Programmed Dendritic Atrophy in Prion Diseases" presented by Dr. Nako Ishikura from the DeArmond Laboratory.

2004 Richard T. Johnson, M.D. Lectureship Award, "The Neurodegenerative Sequence in Prion Diseases" presented at the International Society of NeuroVirology 6th International Symposium in Sardinia, Italy by Dr. Essia Bouzamondo-Bernstein from the DeArmond Laboratory.


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2006 Vice President, International Society of Neuropathology

2003 President, 16th International Congress of Neuropathology

2006 Donation from the Stephen and Patricia Schott Foundation to develop a treatment for prion diseases.

2007 Honored by the AANP Executive Council and Members at Large with an Annual Endowed Lectureship "DeArmond Endowed Lectureship" on Neurodegenerative Diseases for outstanding contributions to the American Association of Neuropathologists and the International Society of Neuropathology and for leadership in neuropathology and basic neurodegenerative research.

2009 Gave the Saul Korey Lecturer at the 2009 Meeting of the American Association of Neuropathologists.

2009 Received the Graduate Citation Award from Drexel University College of Medicine.

2010 Received The Meritorius Service Award from The American Association of Neuroupathologists with an introduction by Stanley Prusiner, M.D., outlining the life and accomplishments of Dr. DeArmond.

KEYWORDS/AREAS OF INTEREST

Neuropathology, Neuroanatomy, Prion diseases, Prions, Creutzfeldt-Jakob disease, Scrapie,

Gerstmann-Sträusler-Scheinker disease, Neurodegenerative diseases, Alzheimer's disease,

frontotemporal dementias, skeletal muscle and peripheral nerve diseases, physiology,

neurochemistry, molecular biology, immunohistochemistry.

PROFESSIONAL ACTIVITIES

CLINICAL

1983-now Attending neuropathologist (consultant) San Francisco General Hospital

1983-now Brain cutting conference, Moffitt-Long hospital for all Creutzfeldt-Jakob and

other prion diseases autopsy and biopsy cases

2000-now Director of a Neuropathology Core to test the efficacy of treatments for

Creutzfeldt-Jakob disease

All of the clinical duties involved supervising neuropathology and anatomic pathology house staff

and medical students. I have been doing this since 1983. I have trained 14 neuropathology

fellows in both clinical and research neuropathology


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SUMMARY OF CLINICAL ACTIVITIES

Until 2009 I spent an average of about 40% of my time with clinical service responsibilities. From

1983 I had the main responsibility for the muscle and nerve diagnostic service. The loss of two

neuropathologists beginning in 1998 left only two of us to cover a very busy diagnostic

neuropathology service. I covered half (2000-2002) and then one third (2003-2003) of the daily

neurosurgical service, which is mostly brain tumor diagnosis and grading. With recruitment of

two primarily diagnostic neurooncology neuropathologists, I covered the daily neurosurgical

service when the other diagnostic neuropathologists were away. I presided over brain cutting at

the Moffitt-Long and San Francisco General hospitals (SFGH), the microscopic review of the

microscopic slides with the residents, and review the final reports. Since 2009 I have presided

over brain cutting and histology sign out at SFGH. For 25 years I have performed all autopsies

on prion disease cases and do the neurohistology review of those cases.

In addition to the clinical neuropathology hospital duties, I have spent much time with The

National Institutes of Health funded studies of human neurodegenerative diseases. Specifically,

I perform and direct quantitative neuropathological studies of Creutzfeldt-Jakob disease cases to

find more efficient methods of early diagnosis and to evaluate whether new drug treatments are

successful or are having adverse effects. These studies are in collaboration with Stanley

Prusiner's laboratory, and Bruce Miller of the UCSF Memory and Aging Center. I have also

reviewed tissues obtained through an Alzheimer's Disease Research Center (ADRC) grant

recently awarded to the Aging and Memory Clinic directed by Bruce Miller to better understand

the frontotemporal dementias and other unusual neurodegenerative diseases. The ultimate goal

of both sets of studies is to find beneficial treatments for these diseases.

PROFESSIONAL ORGANIZATIONS

Memberships

1982 - present Member American Association of Neuropathologists.

1983 - 1987 Member International Society for Neurochemistry.

1996 - present Executive Committee, American Association of Neuropathologists.

2000 - 2001 President of the American Association of Neuropathologists.

2003 - 2006 President of the 2006 International Congress of Neuropathology.

2003 - 2006 Vice- President of the International Society of Neuropathology.

Service to Professional Organizations

1986 - 1986 Member, Awards Committee, American Association of Neuropathologists, Annual Meeting

1987 - 1987 Member, Program Committee, American


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Association of Neuropathologists, Annual Meeting

1988 - 1988 Member, Program Committee, American Association of Neuropathologists, Annual Meeting

1988 - 1988 Chair, Platform Session V (Infectious Diseases), American Association of Neuropathologists, Annual Meeting

1988 - 1988 Member of National Institutes of Aging Neuroscience, Behavior and Sociology of Aging Study Section

1993 - 1993 Chair, Platform Session VIII (Degenerative Diseases), American Association of Neuropathologists, Annual Meeting

1994 - 1994 Chair, Platform Session IX (Degenerative Diseases), American Association of Neuropathologists, Annual Meeting

1995 - 1995 Chair, Platform Session X (Degenerative Diseases), American Association of Neuropathologists, Annual Meeting

1996 - 1996 Chair, Platform Session XI (Degenerative Diseases), American Association of Neuropathologists, Annual Meeting

1996 - present Member of International Scientific Advisory Board, The John Douglas French Alzheimer's Foundation

1996 - present Member of Executive Committee, American Association of Neuropathologists

1997 - 1997 Member, Awards Committee, American Association of Neuropathologists, Annual Meeting

1997 - 1997 Co-chair, Workshop on Prion Diseases, XIIIth International Congress of Neuropathology

1997 - present Ad Hoc Reviewer, National Institutes of Aging Neuroscience, Behavior and Sociology of Aging Study Section

1998 - 1998 Ad Hoc Reviewer, Alzheimer's Disease Program, Department of Health Services, State of California.

2000 - 2000 Chair, Platform Session (Prion Diseases), American Association of Neuropathologists, Annual Meeting, Atlanta, GA.

2000 - 2001 President of the American Association of Neuropathologists

2001 - 2001 Chair of the Committee to restructure the finances of the American Association of Neuropathologists

2001 - 2003 Executive Council and Chair of the Nominating Committee, American Association of


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Neuropathologists

2001 - 2004 Standing Member of the Transmissible Spongiform Encephalopathy Advisory Committee, Food and Drug Administration

2003 - 2006 President of the 16th International Congress of Neuropathology, San Francisco, CA

SERVICE TO PROFESSIONAL PUBLICATIONS

1982 - present Journal of Neuropathology and Experimental Neurology, ad hoc referee

1983 - 1985 Toxicology and Applied Pharmacology, ad hoc referee

1986 - 1986 Science, ad hoc referee

1986 - 1988 Archives of Pathology and Laboratory Medicine, ad hoc referee

1992 - present Neurology; Annals of Neurology, ad hoc referee

2001 - 2007 Editiorial Board, Journal of Neuropathology and Experimental Neurology

INVITED PRESENTATIONS

INTERNATIONAL

1983 International Society for Neurochemistry, (attended meeting)

1986 International Congress of Neuropathology, Stockholm (talks)

1986 International Meeting on Brain Oncology, Rennes (talk)

1987 Ciba Foundation Symposium, "Unconventional Viruses and the CNS," London (talk)

1987 The Royal College of Physicians and Surgeons of Canada Lecturer, 27th Annual Meeting, Canadian Association of Neuropathologists (lecture)

1988 First International Conference on Alzheimer's Disease, Las Vegas (talk)

1988 Australian Rotary Health Research Fund, 3rd Int Conference, Canberra (lecture)

1988 Annual Meeting of Australian-New Zealand Neuropathology Society, Canberra (lecture)

1991 Stanford Centennial Symposium: Neuronal-Astrocytic Interactions: Pathological Implications, Lecture: "New Pespectives in Prion Diseases," Hong Kong (lecture)

1991 Symposium: Prion Diseases in Humans and Animals: "Scrapie prion protein accumulation correlates with neuropathology and incubation times in hamsters and transgenic mice," London (talk)


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1993 Royal Society of London, "Patterns of PrPsc Accumulation in the Brain are Prion Isolate Specific and Independent of Incubation Time" (talk)

1993 The French Foundation for Alzheimer Research: "Parkinsonian Dementia Syndromes" (prion disease lecture)

1994 Symposium: "Prions, Prions, Prions," Lecture: "Trangenics and Neuropathology of Prion Disease," Jefferson Medical College, Philadelphia, PA (talk)

1994 XII International Congress of Neuropathology: "Transgenic Mouse Models Reproduce Prion Disease Variants," Toronto, Canada (talk)

1995 Inclusion Body Myositis Workshop, Univ. Rochester Medical School, Lecture: "Prion Protein Overexpression Myopathy: A Model of Inclusion Body Myositis," West Palm Beach, FL (talk)

1995 International Symposium on Prion Diseases, "Transgenic Mouse Models for Familial Infectious and Sporadic Prion Diseases," Max Planck Institut für Biophysikalische Chemie Am Fassberg, Göttingen, Germany (talk)

1996 Prions and Brain Diseases in Animals and Humans, "The Molecular Basis of Cell Dysfunction in Prion Diseases," Erice, Sicily (talk presented by Dr. Randy Nixon for me because of an injury)

1997 XVI Congreso De La Sociedad Espanola De Microbiologia, "The Conversion of PrPC to PrPSc links neurodegeneration in prion diseases with the propagation of prions" and Emerging and Re-emerging infections, "Prions and spongiform encephalopathies," Barcelona, Spain (lecture)

1997 Conference on Brain Tumours and Alzheimer's Diseases, "Neuron-specific differential glycosylation of PrPC: Relationship to differential targeting of neurons by prion strains," Bali, Indonesia (talk)

1997 XIIIth International Congress of Neuropathology, "Mechanisms of Cell Dysfunction in Prion Disease: PrP Accumulation in Plasma Membrane", Perth, Australia (talk)

1997 Western-Midwestern Meeting, Association of Pathology Chairs, "Molecular Pathogenesis of Transmissible Spongiform Encephalopathies", San Francisco (lecture)

1998 First Annual UCSF-Stanford Current Issues in Anatomic Pathology-1998: "New Variant Creutzfeldt-Jakob Disease", San Francisco (lecture)

1998 Research Rounds, Neurodegeneration Journal Club, University of California, San Diego, "Prion Diseases" (lecture)


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1998 International Workshop on Conformational Diseases, "Brain Region PrPC Heterogeneity: Implications for Selective Targeting of Neurons by Prion Strains", Ein-Boqeq, Dead Sea, Israel (lecture)

2000 World Alzheimer Congress, "Neuropathology of Prion Diseases", Washington, D.C. (talk)

2001 International Meeting on Diseases of Protein Conformation, "Prion Protein Conformation and the Pathogenesis of Dysfunction, Degeneration and Death of Neurons", Sigrid Juselius Foundation, Helsinki, Finland (lecture)

2001 Presidential Symposium, American Association of Neuropathologists, "Molecular Pathogenesis of Neurodegeneration in Prion Diseases", Chicago (lecture)

2001 9th International Congress of Toxicology, "Prion Protein Diseases: the Mad Cow Disease and New Variant CJD Crises", Brisbane, Australia (lecture)

2001 "Prion Protein Diseases: Pathogenesis of Neurodegeneration and Methods of Disinfecting in Medical Practice", University of Melbourne, Melbourne, Australia (lecture)

2002 "The Journey to Discovery of Prions", 16th Annual Peter Lampert Lecture, University of California, San Diego (lecture)

2002 International Symposium on Emerging Infectious Diseases and Bioterrorism: Regional Threat, Global Impact, "The Threat Posed by New Prion Diseases", University of California, Davis (lecture)

2002 35th Annual Advances and Controversies in Clinical Pediatrics, "Mad Cow Disease: Implications in Pediatrics". San Francisco, CA (lecture)

2003 Plenary Lecturer on Mechanisms of Neurodegeneration in Prion Diseases, International Prion Conference, Munich, Germany (lecture)

2006 Plenary Lecturer, "Activation of Notch-1 Signaling Pathways Link PrPSc Accumulation with Dendritic Atrophy and Hyper-reactive Astrocytic Gliosis." 16th International Congress of Neuropathology (lecture).

2008 "The Pathogenesis of Alzheimer's Disease and its Relationship to Other Neurodegenerative Diseases." Best Practices in the Continuum of Care: Advances in Alzheimer's Disease Management. Office of Continuing Medical Education, College of Medicine, University of Arkansas, Little Rock, Arkansas. April 16, 2008 (Lecture).

2008 "Pathology and Pathogenesis of Human TSEs." International Workshop: Human and Animal TSE, Grado


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Italy, 26-28 June, 2008 (Lecture).

2008 "Neuropathology of Parkinson Disease" Parkinson's Diseasea and Prions Meeting, William J. Rutter Community Center, UCSF, October 2008.

2009 "Are Alzheimer's Disease and Parkinson's Disease Prion Diseases?" University of Texas, San Antonio, Spring 2009 Neurobiology Seminar Series Guest Speaker.

2009 Saul Korey Lecturer at the 2009 Meeting of the American Association of Neuropathologists.

NATIONAL

1969 Federation of American Societies for Experimental Biology (talk)

1971 Federation of American Societies for Experimental Biology (talk)

1979 American Association of Neuropathologists, 1988 (1 talk, 2 posters)

1979 Winter Conference on Brain Research (lecture)

1980 American Association of Neuropathologists (talk)


1981 Veterans Administration Regeneration Research Symposium, Portland (lecture)


1984 American Association of Neuropathologists (talk and poster)

1984 American Society for Neurochemistry (symposium lecturer)

1984 Veterans Administration Regeneration Research Symposium, Mt. Hood, OR (paper)

1985 American Society for Cell Biology (poster), 1987

1985 SUNY, Stonybrook, Anatomical Science Lecture

1985 UCLA, Pathology Research Lecture

1985 American Association of Neuropathologists (2 papers)

1986 University of Pennsylvania Medical School, Visiting Professor, Neuropathology (lecture)

1986 American Association of Neuropathologists (talk and poster)

1986 American Society for Virology (talk)

1986 The John Douglas French Foundation for Alzheimer's


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Disease (workshop)

1986 American Society for Neurochemistry (symposium speaker)

1987 American Association of Neuropathologists (2 papers)

1988 Sterling Research Group, Malvern, PA (lecture)

1989 University of California, Los Angeles, Pathology Grand Rounds, "Dementia and Unusual Infections of the Nervous System" (lecture)

1990 National Multiple Sclerosis Society Workshop, "Role of Astrocytes in Inflammatory Demyelination," lecture, "GFAP Synthesis," Galveston, TX (lecture)

1991 Society for Experimental Neuropathology Symposium, "The Prion Hypothesis: Support from Transgenic Mice and Cell Biology Studies," Seattle, WA (lecture)

1992 Sutter Neuroscience Center Annual Symposium, "Prions: Relationship to CNS Disease and Safety Issues," Sacramento, CA (lecture)

1992 Keystone Symposium: Advances in Understanding Neurodegenerative Disorders, "Brain Lesions in Spongiform Encephalopathies," Big Sky, MT (lecture)

1992 Cold Spring Harbor Symposium: Molecular Biology of Aging, "Scrapie Prion Replication is Brain Region Specific in Transgenic Mice and Hamsters," Cold Spring Harbor, NY (lecture)

1993 Visiting Professor, University of Colorado School of Medicine, "Pathogenesis of Prion Diseases," Denver, CO (lecture)

1993 American Association of Neuropathologists, "The Sites of PrPSc Deposition in the Brain are Prion Strain Specific," Salt Lake City, UT (talk)

1993 Visiting Professor, McLaughlin Research Institute, "The Molecular Pathogenesis of Prion Disorders in Transgenic Mice," Great Falls, MT (lecture)

1995 Neurofest '95 "Diseases of the Aging Brain," Lecture: "Prion Diseases," SUNY Syracuse (lecture)

1995 American Association of Neuropathologists, Annual meeting, Chair, "Degenerative Diseases, Prions and ALS," San Antonio, TX (talk)

1995 Banbury Center Conference on Molecular Biology of Prions and Pathology of Prion Diseases, "Transgenic Models of Prion Disease Pathogenesis," Cold Spring Harbor, NY (lecture)

1996 Cold Spring Harbor Laboratory 61st Symposium on


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Quantitative Biology, Function and Dysfunction in the Nervous System, "Pathogenic Mechanisms in Infectious Prion Diseases: Cell Biology Perspective," Cold Spring Harbor, NY (lecture)

1996 The Lucien J. Rubinstein Visiting Research Lectureship in Neuropathology, "Molecular Pathogenesis of Prion Diseases," & "Muscle Dysfunction in Prion Diseases," Univ. Virginia Medical School, Charlottesville, VA (2 lectures)

1996 The Southern California Virology Association, "From Kuru to Mad Cow's Disease", San Diego, CA (lecture)

1997 Experimental Biology '97 "Prion PrPSc accumulation causes cell dysfunction," New Orleans, LA (lecture)

1997 American Association of Neuropathologists, Special Course: Prion Protein Diseases, "Mechanisms of Cell Dysfunction in Prion Diseases," Pittsburgh, PA (lecture)

1998 17th Annual Nobuhisa Baba Memorial Lecturer, "Prions: Invaders from Innerspace", Department of Pathology, Ohio State University. Also, visiting Professor of Pathology giving two other lectures: "Spectrum of Neuropathological Phenotypes in Human Prion Diseases" and "Research Productivity Requires More than Good Research Questions" (2 lectures)

1999 32nd Annual Recent Advances in Neurology, sponsored by UCSF Department of Neurology, "Kufs Disease", San Francisco (lecture)

1999 Departments of Pathology and Laboratory Medicine Grand Rounds, "Pathogenic Mechanisms in Dementing Diseases", UCSF, 2/24/99 (lecture)

1999 Scientific Symposium: Recent Progress in Anatomic Pathology, American Association of Clinical Pathology National Meeting, "Update on Prion Diseases (Will "Mad Cow" Disease Spread to Humans?), Orlando, FL, 4/12/99 (lecture)

2000 Focus Group: Transgenic Models in the Nervous System, "Modulation of the disease phenotype in transgenic mice expressing different prion protein constructs", Annual Meeting of Experimental Biology, San Diego, CA, 4/16/00 (lecture)

2001 Oregon Health Sciences University, Department of Pathology, "The Molecular and Biological Basis of Creutzfeldt-Jakob Disease Treatment" (lecture)

2002 Annual Meeting of Experimental Biology, San Diego, CA "Modulation of the Disease Phenotype in Transgenic Mice Expressing Different Prion Protein Constructs" (lecture)


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2006 New York Academy of Sciences, NY "Notch Signaling in Prion Disease" (lecture)

2007 Torrey Pines Therapeutics, San Diego, CA: "Gamma Secretase Inhibitor and Quinacrine Work Synergistically to Reduce PrPSc and Prevent Dendritic Degeneration in Mice with Scrapie. (lecture)

REGIONAL AND OTHER INVITED PRESENTATIONS

1983 UCSF, Pathology, Resident Teaching Grand Rounds


1984 UCSF, Neurosurgery, Grand Rounds

1984 UCSF, Neurology, Grand Rounds

1985 Northern California Society for Electron Microscopy, Palo Alto (invited lecturer)

1985 Palo Alto VA/Stanford University, Pathology Research Seminar


1985 Current Issues in Anatomic Pathology, "Identification of Myopathies by Combined Morphological Techniques," San Francisco (UCSF)

1985 Stanford University Medical School, Neurology Grand Rounds

1986 Current Issues in Anatomic Pathology, "New Concepts: Pathogenesis of Alzheimer's Disease and Other Dementias," San Francisco (UCSF)

1986 UCSF, Neurology Grand Rounds

1986 The John Douglas French Foundation for Alzheimer's Disease, Workshop, "The Neuropathology of Alzheimer's Disease"

1987 College of American Pathologists Seminar, "Update on Dementing Illnesses," San Francisco, CA

1987 UCSF, Neurology Residents Rounds

1987 Stanford University Medical Center, Neurology Grand Rounds

1989 International Congress of Pathology, "Pathophysiology of Spongiform Encephalopathies," shared lecture with Dr. Stanley Prusiner, San Francisco, CA

1989 Stanford University Medical Center, Pathology Grand Rounds, "Molecular Pathology of Prion Disease," Stanford, CA

1992 San Francisco Neurological Society Annual Meeting,


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"Neuropathological Issues in AIDS," Monterey, CA

1992 UCSF Department of Pathology, 8th Annual Current Issues in Anatomic Pathology: "Science Lecture: Alzheimer's Disease and Jakob-Creutzfeldt Disease--New Insights and Significant Advances," San Francisco, CA

1992 St. Josephs' Medical Center, Scientific Rounds: "Prion Diseases and Related Safety Issues", Stockton, CA

1992 American Aging Association, 22nd Meeting, Minisymposium (Neurobiology of Aging and Alzheimer's Disease): "Multiple Pathogenic Mechanisms in Prion Diseases", San Francisco, CA

1996 Commonwealth Club of San Francisco, "Mad Cow's Disease, Scrapie, and Prions," San Francisco, CA

1996 Centeon/UCSF Prusiner Research Conference on Prions, "Prions and PrPSc in Human Brains," Rutherford, CA

1997 Centeon/UCSF Prusiner Research Conference on Prions, "Neuronal Targeting in Prion Diseases," Monterey, CA

1997 Western-Midwestern Meeting of the Association of Pathology Chairs, "Molecular Pathogenesis of Transmissible Spongiform Encephalopathies", San Francisco, CA

2003 California Society for Histotechnology, 27th Annual Symposium/Convention, "Neurohistology's Role in Understanding the Pathogenesis of Prion Diseases", Monterey, CA.

CONTINUING EDUCATION COURSES ATTENDED

1980 Am. Assoc. of Neuropathologists, Developmental Neuropathology

1981 Am. Assoc. of Neuropathologists, Immunocytochemistry in Neuropathology

1982 Am. Assoc. of Neuropathologists, Forensic Neuropathology

1982 University of California, Davis

1984 Am. Assoc. of Neuropathologists, Quantitative Neuropathology and Human Neurogenetics

1985 Am. Assoc. of Neuropathologists, Muscle Pathology, Cold Spring Harbor Symposium

1993 Am. Assoc. Neuropathologists Annual Mtg., "The Sites of PrPSc Deposition in the Brain are Prion Strain Specific"

1993 Royal Society of London, "Patterns of PrPSc Accumulation in the Brain are Prion Isolate Specific and Independent of Incubation Time"

1993 The French Found. For Alzheimer Research, "Parkinsonian Dementia Syndromes"


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1994 XII Int'l Congress of Neuropathology, "Transgenic Mouse Models Reproduce Prion Disease Variants"

1994 Jefferson Medical College Symposium, "Prions, Prions, Prions", Workshop, Univ. Rochester Med. School

1995 Neuromuscular Symposium, Florida, Inclusion Body Myositis

1995 Neurofest '95 SUNY, "Diseases of the Aging Brain"

1995 Am. Assoc. of Neuropathologists Annual Meeting, Chair, "Degenerative Diseases, Prions and ALS"

1996 Am. Assoc. Neuropathologists, Canada

1997 Am. Assoc. of Neuropathologists, Transmissible Spongiform Encephalopathies

1997 XIIIth Int'l Congress of Neuropathology

1997 Conference, Bali, Indonesia, Brain Tumors and Alzheimer's Disease

1997 Microbiology Conference, Spain, Emerging and Re-emerging Infections Disorders

1997 Western- Midwest Assoc. Pathology Chairs

1997 First Annual UCSF-Stanford Meeting, Current Issues in Anatomic Pathology

1998 Int'l Workshop, Dead Sea, Israel, Conformational Diseases

1998 17th Annual Nobuhisa Baba Memorial Lecture

1998 Robert E. Stowell Lectureship, UC Davis

1999 Am. Assoc. Neuropathologists Annual Mtg., Infectious Diseases of the Nervous System

1999 Microscopy and Microanalysis Society AIDS Dementia Complex Symposium

2000 American Academy of Neurology

2000 Am. Assoc. Neuropathologists Annual Mtg.

2001 Am. Assoc. Neuropathologists Annual Mtg., Developmental Neurobiology

2002 Am. Assoc. Neuropathologists Annual Mtg., Microglia in Neuropathology

2003 Int'l Symposium, München, Germany, Prion Diseases

2003 Am. Assoc. Neuropathologists Annual Mtg., Magnetic Resonance Imaging of Neurological Disease

2004 Am. Assoc. Neuropathologists Annual Mtg., Advances in the Diagnosis and Biology of Brain Tumors

2004 Frontotemporal Dementia Symposium

2005 Am. Assoc. Neuropathologists Annual Mtg., Cellular and Molecular Pathology of the CNS in Epilepsy and Neuropsychiatric Disorders

2006 International Congress of Neuropathology, Attendended 5 minisymposia and 4 workshops

2007 Am. Assoc. Neuropathologists Annual Mtg., Update on Brain Tumors: Prognostic Markers and Controversies in Diagnosis

2008 Am. Assoc. Neuropathologists Annual Mtg., Molecular Embryology to Malformations


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2009 Am. Assoc. Neuropathologists Annual Mtg., Recent Advances that Impact Research and Clin Practices

2010 Am. Assoc. Neuropathologists Annual Mtg., Neurofibromatosis and Related Diseases

GOVERNMENT AND OTHER PROFESSIONAL SERVICE

1996 - present John D. French Foundation, Member of Scientific Advisory Committee

1991 - 2001 National Institutes of Aging Neuroscience, Behavior, and Sociology of Aging Review Committee, Ad Hoc and Study Section member

2001 - 2004 Federal Drug Administration, member of the Transmissible Spongiform Encephalopathy Advisory Committee

1999 - 2000 President of the American Association of Neuropathologists

2003 - 2006 Vice President of the International Society of Neuropathologists

2003 - 2006 President of the 2006 International Congress of Neuropathologists (theme: Integration of Neuropathology with Drug Development)

2006 - present McLaughlin Research Institute, Scientific Advisory Committee

UNIVERSITY AND PUBLIC SERVICE

UNIVERSITY SERVICE

UCSF SYSTEM-WIDE

1986 - 1987 Committee on Rules and Jurisdiction, San Francisco Division

1987 - 2000 Member of Faculty Promotion Committee

1999 - present Executive Committee, the Institute for Neurodegenerative Diseases

DEPARTMENTAL SERVICE

1983 - 1998 Co-Director, Neuropathology Unit

1983 - 1985 Laboratory Safety Committee

1984 - 1984 Search Committee for Chief Histotechnologist

1984 - 1984 Program Committee, Current Issues in Anatomic Pathology

1987 - 1990 Secretary, Department of Pathology Research Caucus


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1987 - 1990 Residency Selection Committee

1992 - 2001 Director of Neuropathology Training Programs

1992 - 2001 Chair, Neuropathology Faculty Search Committee

1998 - 2001 Director of Neuropathology Unit

PUBLIC SERVICE

1979 - 1983 Coach, AYSO Soccer, Palo Alto, CA

1984 - 1984 Alzheimer's Disease Research Center, ad hoc Review Committee, National Institutes of Aging (Dr. Folstein, Chairman), Bethesda, MD

6/20-23

1985 - 1985 Alzheimer's Disease Research Center, ad hoc Review Committee, National Institutes of Aging (Dr. Les Weiner, Chairman), Bethesda, MD

6/5-8

1986 - 1986 Site visit to Institute for Basic Research, Staten Island, NY, ad hoc committee, National Institute of Child Health and Human Development (Dr. David Wenger, Chairman)

11/29-12/1

1990 - 1990 Site visit to Rush-Presbyterian-St. Luke's Medical Center, Chicago, IL, ad hoc committee, National Institutes of Aging (Dr. Helena Chiu, Chairperson)

5/21-22

1990 - 1990 Member, National Institute of Aging study section: Neuroscience, Behavior and Sociology of Aging Review Committee A

1993 - present Executive Committee, National Science Advisory Board, The John Douglas French Foundation for Alzheimer's Research

2001 - present Food and Drug Administration, Transmissible Spongiform Encephalopathy Advisor Committee, standing member.

2003 - 2003 Testifying before the California Legislature Committee on Agriculture and Water Resources about bovine spongiform encephalopathy (BSE).

TEACHING AND MENTORING

TEACHING

FORMAL SCHEDULED CLASSES FOR UCSF STUDENTS

Qtr Academic Yr Course Number and Title Teaching

Contribution Units

Class Size

W 1983 - Pathology 102 Lecturer


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Qtr Academic Yr Course Number and Title Teaching

Contribution Units

Class Size

present

W 1983 - present

Pathology 102 Laboratory Instructor

F 1984 - 1985 Medical Illustration Lecturer

F 1988 - present

Pathology 126, 127, 135, 101A Lecturer

F 1997 - 2001 Pathology 1

S 2002 - 2009 Brain, Mind and Behavior for Medical Students

Lecturer, Small Group Instructor

INFORMAL TEACHING

- Reading neurosurgical slides

- Reading muscle and nerve biopsies

- Reading neuropathology autopsy slides

- Autopsy procedures and brain cutting conferences

- Clinical and basic research techniques

TEACHING NARRATIVE

NOTE: I was recruited to UCSF Pathology in 1983 by Dr. Ed Smuckler, then Chair of Pathology,

to develop an integrated clinical and investigative neuropathology program. Over a sixteen year

period, Dr. Richard Davis, who was recruited in 1980 because of his International reputation as a

diagnostic neuropathologist, and I managed to build one of the top three Neuropathology

Training Programs in the USA. I brought solid investigative neuropathology in the biology of

astrocytes and glial neoplasms form my years at Stanford and made the transition to studies of

the pathogenesis of neurodegeneration and reactive astrocytic gliosis in prion diseases in 1984

at the request of Stanley Prusiner. Dr. Davis recruited fellows until 1990. Thereafter, I became

Director of the Neuropathology Training Program.

I encouraged Neuropathology fellows to work in Neuroscience-related labs at

UCSF of their own choice because I believe a trainee will be more successful if their research is

in an area of interest to them. Seven of fourteen neuropathology fellows have done all of their

basic research in my Neuropathology Research Laboratory, mostly on prion diseases. Six

chose to work in other outstanding laboratories at UCSF. One, Dr. Bollen, did not participate in

basic neuropathology research because he planned to have a diagnostic surgical pathology,

neuropathology, and clinical pathology career. All of the fellows ultimately became American

Board of Pathology certified in Neuropathology. All but one added at least one publication to

their CV as a result of their research experience.


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The goal of research training in my laboratory has been to acquaint the fellow

with laboratory investigative techniques. But, more importantly, my goal has been to teach them

how to ask the most relevant and testable questions. This is done via formal lab meetings,

review of data, helping with writing manuscripts and grants, and casual discussions on and off

campus. The number of hours spent teaching and mentoring each fellow is difficult to assess

because it is an ongoing process occurring on a daily basis.

MENTORING

PREDOCTORAL STUDENTS SUPERVISED OR MENTORED

Dates Name Program or School Role Current Position

1984 - 1986 Theresa Trebaol, William Dutton

UCSF School of Medicine

Medical Illustration Teacher

Unknown

1984 - 1986 Bernie Kida Medical Illustration Student

Suggested illustrations, advised on content, and judged accuracy

Medical Illustrator

1984 - 1986 Beth Collins, Rick Adler, Bernie Kida, Ken Richardson

Medical Illustration Students

Suggested illustrations, advised on content, and judged accuracy

Unknown

1992 - 1992 Alexander Kon

Medical Student, McGill University

Summer elective program. Neuropath/research methods for neurodegenerative diseases

Associate Professor UC Davis

1991 - 1992 Russell Bowler, MD with Thesis because of work in my Lab

UCSF Medical Student

Studied prion protein receptors and distributions of PrPSc by a then new technique

Internship and residency in Internal Medicine, UCSF

1991 - 1992 Arun Amar, MD with Thesis because of work in my Lab

UCSF Medical Student

In our ongoing studies with the BTRC at UCSF, Arun developed an in vitro assay in my lab to measure brain tumor invasion

Neurosurgery training at USC. Neurosurgery faculty at Yale University Medical School

1992 - 1992 Audrey Lee UCSF Medical Student

In my lab, participated in studies of PrPSc distribution in

Associate Clinical Professor Department of Clinical Pharmacy UCSF


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Dates Name Program or School Role Current Position

brains of scrapie infected transgenic mice

1993 - 1993 Anne Emily Ninchak-Casey, M.D.

UCSF Medical Student II

Anne performed complex two-dimensional gel studies of PrPC charge isomers as a function of different brain regions. She showed that PrPC from each brain region has a different set of carbohydrate trees.

UCSD Neurology Resident

1993 - 1993 Jason Vanclef

Senior Chemistry Major, Cal Poly, San Luis Obispo

Compared intracellular calcium fluxes in scrapie infected ScN2a cells and uninfected N2a cells following exposure of the cells to bradykinin.

Employed by the Coroner's Office, Los Angeles, CA

1993 - 1993 Jennifer Sanchez-Salazar

Whittier College Helped with a variety of neurohistological and immunohistochemical projects in my lab

Severe auto accident un 1993. Current position unknown.

1998 - 1999 Ignatius (Nate) Gerodias

B.S. in Biology, University of Pacific

Especially diligent and cheerful helper in my Neurohistology Laboratory

2005 began faculty position at Children's Memorial Hospital, Chicago.

1999 - 2000 Albert Tsai Recent Graduate, UC Berkeley

Helped a postdoc in my lab immunostain serial sections of scrapie-infected and control hamster brains for calcium binding proteins and GABA.

Medical Student, Boston University

2006 - 2006 Azucena Lemus

B.S. Chemistry, University of Bogata, Columbia

Helped with all routine neurohistological tecniques and learned histoblot and immunohistochemistry

Staff Research Assistant, DeArmond Neuropathology Research Laboratory


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POSTDOCTORAL FELLOWS AND RESIDENTS DIRECTLY SUPERVISED OR MENTORED

Dates Name Fellow Faculty Role Current Position

1983 - 1986 Hans Kretzschmar MD

Neuropathology Fellow, Prion research in my lab

Suspervised research year

Director, Institute of Prion and Neurodegenerative Diseases, Munich, Germany

1984 - 1987 James T. Rutka, MD, PhD

Exp. Pathology at UCSF. Research performed in my lab and others at UCSF for studies of molecular mechanisms in gliomas

Mentored Ph.D. in Exp Pathology at UCSF

Professor and Chair Department of Neurosurgery, The Hospital for Sick Children, Toronto, Canada

1986 - 1988 Howard Charman, MD

Neuropathology Fellow,Prion research in my lab

Supervised research

Private practice pathology & neurpathology

1986 - 1988 Michael Gonzales, M.D

Research Fellow in my prion disease research lab. effects of prions on astrocytes

Supervised research year

Director of Neuropathology, Royal Melbourne Hospital, Victoria, Australia

1988 - 1990 Frank Coufal, M.D.

Research Fellow in my prion disease research lab, search for prion protein receptors

Supervised research year

Neurosurgery residency, UCSD

1988 - 1991 Klaus Jendroska, M.D.

Research Fellow in my prion disease research lab, first quantitative study of PrPSc kinetics in hamster scrapie

Supervised research yeara

Neurologist, Professor, Department of Neurology, Virchow-Hospital, Charite, Berlin, Germany

1988 - 1990 Andrew W. Bollen, MD

Neuropathology Fellow, no bench research training

Neuropathology teacher

Professor of Pathology and Neuropathology, and Director of the Neuropathology Unit, UCSF

1989 - 1992 Jack M. Raisanen, MD


Supervised research

Private practice pathology & neuropathology

1991 - 1992 Arun Amar Medical Student Supervised research

Clinical Instructor, Neurosurgery, Stanford University (Medical Center)

1991 - 1992 Russel Bowler

Medical Student Supervised research

Asst Prof, Dept. Medicine, Nat'l Jewish Medical and Research Ctr., Denver, CO

1991 - 1993 Kenneth D. Aldape, MD

Neuropathology Fellow, Brain tumor research in Mark Israel's lab in the BTRC

Supervised research, NP teacher

Associate Professor of Neuropathology, M.D. Anderson Cancer Center, Houston, TX


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1992 - 1995 Rosalie M. Uht, MD, PhD

Neuropathology Fellow, Steroid-neurodegeneration research with J.F, McKelvy in Metabolic Res Unit, UCSF

Supervised research. NP teacher

Assistant Professor of Pathology and Biochemistry and Molecular Genetics, University of Virginia Medical School, Charlottesville, VA

1993 - 1995 Kondi Wong, MD, Cpt. USAF


Supervised research year, NP teacher

Chief, Division of Neuromuscular Pathology, Armed Forces Institute of Pathology, Washington, DC

1994 - 1997 Randal R Nixon, MD, PhD



Associate Professor and Director of Neuropathology, University of Oregon Health Science Center, Portland, OR

1995 - 1997 Eric J. Huang, MD, PhD

Neuropathology Fellow, Postdoctoral fellow with Louis Reichardt in Developmental Neurobiology


Assistant Professor of Neuropathology, San Francisco V.A. Medical Center

1996 - 1997 Michael Alan Shea, M.D.

Research Fellow in my prion disease research lab

Supervised research

General Practice, Medicine

1997 - 1999 Martha L. Simmons, MD,PhD



Formerly Clinical Assistant Professor, Neuropathology, UCSF; Neuropathology course director

1997 - 1999 Jie Zhou, PhD

Dr. Zhou was a postdoctoral fellow and Assistant Research Cell Biologist under Dr. William Mobley in Neurology at UCSF. I paid her salary and gave her lab space for two years because her work on TrkB receptors in membrane rafts, which was relevant to my own research

Supervised research

Attorney at law working in the offices of Morrison & Foerster, Palo Alto, CA

1997 - present Essia Bouzamondo, Ph.D.

Post Doctoral Fellow in my lab who has performed many studies showing a stereotypical progression of neuronal dysfunction

Supervised research

Associate Researcher, Department of Pathology, UCSF


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and degeneration in prion diseases

1999 - 2003 David Gaskin, M.D.

Post Doctoral Neuropathology Research Scholar, worked in my lab with quantitative studies of neuropathological changes in human prion diseases

Mentor Assistant Professor, Dalhousie University, IWK Children's Health Center, Department of Pathology, Nova Scotia, Canada

1999 - 2002 Jane H. Uyehara-Lock, MD


Supervised research, NP teacher

Pathologist, Hawaii Permanente Medical Group, Moanalua, HI; Alzheimer's disease research, Pacific Health Research Institute

2001 - 2003 William McDonald, MD

Neuropathology Fellow, Brain tumor research with Dennis Dean in the BTRC at UCSF


Anatomic pathologist and neuropathologist, Pathology Associates, St. Paul, MN

2001 - 2005 Nako Ishikura, M.D., Ph.D.

Post Doctoral Research Fellow in my lab studying gene expression in prion diseases

Supervised research

Clinical Psychiatrist, Takatsuki Psychiatric Hospital, Tokyo, Japan

2001 - present Ania Gheyara (Anieszka (Sheila) Niewmierzycka), MD, PhD

Neuropathology Fellow, helped with short term prion disease study in my lab, now in Louis Reichardt's Lab, Neuroscience Department

Supervised research, Neuropathology teacher

Neuropathology Fellow, UCSF Pathology

2002 - present Marta Margeta, MD, PhD

Neuropathology Fellow, developmental neurobiology research with Lilly Jan in the Department of Physiology & Biochemistry/Biophysics

Mentor; neuropathology teacher

Clinical Instructor, UCSF Pathology (Neuropathology) Department

2004 - present William Seeley, M.D.

Neurology Fellow, Memory and Aging Clinic; Frontotemporal dementia research project

Mentor; supervise research

Clinical Instructor, UCSF Department of Neurology

2006 - 2008 Thomas Tousseyn, M.D.

Visiting Post-Doctoral Scholar from Belgium to receive general training in

Mentor; supervisor research


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Neurodegenerative Diseases

- POST DOCTORAL TRAINEES FROM STANLEY PRUSINER'S LABORATORY WHO RECEIVED NEUROPATHOLOGY AND NEUROANATOMY TRAINING IN MY LABORATORY

1984 - 1986 David Bolton 1984 - 1991 Michael

McKinley

1984 - 1985 Paul Benheim

1984 - 1985 Ronald Barry 1985 - 1986 Kevin Boylan 1985 - 1987 Ruth Gabizon 1986 - 1989 Karen Hsiao 1986 - 1992 Daniel

Lowenstein

1986 - 1992 Albert Taraboulos

1989 - 1992 Rolf Hecker 1990 - 1993 Glenn Telling 1991 - 1993 Fruma

Yehiely

1991 - 1996 Patrick Bosque

1993 - 1996 Jorg Tatzelt 1993 - 1999 David Peretz 1995 - 1998 Surchai

Supattapone

1995 - 1998 Martin Vey 1996 - 1998 Richard

Moore

1997 - 2000 Veronique Perrier

1999 - 2001 Gerold Schmitt-Ulms


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1999 - 2002 Chungsuk Ryou

2004 - 2008 Sam Saghafi (Vishu Lingappa Lab)

2005 - 2010 Erdem Tamgünay

2005 - 2010 David Colby 2006 - 2010 John Price 2007 - 2009 Misol Ahn 2007 - present Jan Stöhr 2008 - present Joel Watts

OTHER VISITING FACULTY SUPERVISED

1996 - 1996 Krister Kristensson, M.D. was a neuropathologist at the Karolinska Institute, Huddinge, Sweden, who spent a sabbatical year in my lab. He began a series of studies we were to continue after he left that tested whether or not accumulation of PrPSc in ScN2a cells altered transmembrane signaling.

1997 - 1998 Alfonso Carlos Neves, M.D., Ph.D. Visiting Scholar in my prion disease research lab who studied prion contamination of blood.

Neurologist, Sao Paulo Hospital, Sao Paulo, Brazil.

TEACHING AND MENTORING AIDS

1983 Revised Neuropathology portion of Pathology 102 syllabus (37 pages) (revised annually). 1983-now Decided content and method of teaching neuropathology section of Pathology 102 1984-85 Prepared new pathology specimens for Pathology 102 1987-88 Revised my textbook of neuroanatomy "Structure of the Human Brain" used by most medical and graduate students in the U.S.A. and abroad. 1987-95 Integrating Neuropathology and Introduction to Clinical Medicine lectures


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TEACHING AND MENTORING AWARDS AND NOMINATIONS

Nominated on several occasions for teaching awards for my neuropathology lectures to medical students.

SUMMARY OF TEACHING AND MENTORING HOURS

2010 - 2010 262 total hours of teaching (including preparation)

Formal class or course teaching hours: 0 hours

Informal class or course teaching hours: 0 hours

Mentoring hours: 0 hours

2011 - 2011 262 total hours of teaching (including preparation)

Formal class or course teaching hours: 0 hours

Informal class or course teaching hours: 0 hours

Mentoring hours: 0 hours

2012 - 2012 Total anticipated hours of teaching: 200 hours

RESEARCH AND CREATIVE ACTIVITIES

RESEARCH AWARDS

CURRENT

P01 AG02132 (PI) 01/01/1986 - 01/31/2014

NIH/NIA

Degenerative and Dementing Diseases of Aging

P01 AG021601 (PI) 06/01/2003 - 05/31/2014

NIH/NIA

Novel Therapeutics for Prion Diseases

Schott Foundation-DeArmond (PI) 04/01/2006 - 03/31/2016

Stephen and Patricia Schott Foundation Fund

P01 NS41997 (PI) 07/01/2007 - 06/30/2012

NIH

Genetics of Prion Susceptibility in Vitro: PrPSc Activation of Notch-1 Signaling Pathways as a Cause of Synaptic


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Degeneration.

P01 AG010770 (PI) 04/01/2011 - 03/31/2016

NIH

Patholgenic Mechanisms in Prion Diseases.

R01 AG033017 (PI) 03/15/2009 - 02/28/2014

NIH/NIASelective Vulnerability in Frontotemporal Dementia.

PAST

R01 NS 22786 (PI) 06/01/1990 - 06/30/2000

NIH/DPHS

Transmissible and Genetic Neurodegenerative Diseases

R01 NS 14069 (PI) 01/01/1988 - 05/31/2002

NIH/NINDS

CNS degeneration of Novel Pathogens

RG 0063/1994B (PI) 07/04/1994 - 08/30/1997

HFSP

Function and Conformation of Prion Protein

P01 NS41997 (PI) 09/30/2001 - 07/31/2006

NIH/NINDS

Functional Genetics of Susceptibility to Prions

N01 NS02328 (PI) 09/29/1999 - 09/28/2007

NIH/NINDS

Development of Assay for Creutzfeldt-Jakob Diseases

AG023501 (ADRC) 04/01/2004 - 05/31/2009

NIH/DHHS

New Approaches to Dementia Heterogeneity

P01 AG10770 (PI) 07/01/1992 - 03/31/2010

NIH/NIA


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Pathogenic Mechanism in Prion Disease

PEER REVIEWED PUBLICATIONS

1. DeArmond SJ, Fusco MM. The effect of preoptic warming on the arousal system of the

mesencephalic reticular formation. Exp Neurol, Issue: 3. 33: 653-70, Dec/1971.

2. DeArmond SJ, Eng LF, Rubinstein LJ. The application of glial fibrillary acidic (GFA) protein

immunohistochemistry in neurooncology. A progress report. Pathol Res Pract, Issue: 4. 168: 374-94, 1980.

3. Brant-Zawadzki M, Anderson M, DeArmond SJ, Conley FK, Jahnke RW. Radiation-induced

large intracranial vessel occlusive vasculopathy. AJR Am J Roentgenol, Issue: 1. 134: 51-5, Jan/1980.

4. DeArmond SJ, Deibler GE, Bacon M, Kies MW, Eng LF. A neurochemical and

immunocytochemical study of P2 protein in human and bovine nervous systems. J Histochem Cytochem, Issue: 12. 28: 1275-85, Dec/1980.

5. DeArmond SJ, VandenBerg SR, Herman MM. Neural differentiation in the OTT-6050 mouse

teratoma: effects of intracerebral environment on the neural differentiation of embryoid bodies. Virchows Arch A Pathol Anat Histol, Issue: 1. 393: 39-52, 1981.

6. VandenBerg SR, Hess JR, Herman MM, DeArmond SJ, Halks-Miller M, Rubinstein LJ.

Neural differentiation in the OTT-6050 mouse teratoma. Production of a tumor fraction showing melanogenesis in neuroepithelial cells after centrifugal elutriation. Virchows Arch A Pathol Anat Histol, Issue: 3. 392: 295-308, 1981.

7. VandenBerg SR, Chatel M, Griffiths OM, DeArmond SJ, Pappas C, Herman MM. Neural

differentiation in the OTT-6050 mouse teratoma. Production of a tumor fraction restricted to stem cells and neural cells after centrifugal elutriation. Virchows Arch A Pathol Anat Histol, Issue: 3. 392: 281-94, 1981.

8. Eng LF, DeArmond SJ. Glial fibrillary acidic (GFA) protein immunocytochemistry in

development and neuropathology. Prog Clin Biol Res, 59A: 65-79, 1981.

9. DeArmond SJ, Siegel MW, Dixon RG, Eng LF. Post-embedding immunoperoxidase staining

of glial fibrillary acidic protein for light and electron microscopy. J Neuroimmunol, Issue: 1. 1: 3-15, Mar/1981.

10. Junck L, Enzmann DR, DeArmond SJ, Okerlund M. Prolonged brain retention of contrast

agent in neonatal herpes simplex encephalitis. Radiology, Issue: 1. 140: 123-6, Jul/1981.

11. Sobel RA, DeArmond SJ, Forno LS, Eng LF. Glial fibrillary acidic protein in hepatic

encephalopathy. An immunohistochemical study. J Neuropathol Exp Neurol, Issue: 6. 40: 625-32, Nov/1981.


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12. DeArmond SJ, Fajardo M, Naughton SA, Eng LF. Degradation of glial fibrillary acidic protein

by a calcium dependent proteinase: an electroblot study. Brain Res, Issue: 2. 262: 275-82, Mar/7/1983.

13. Bendheim PE, Barry RA, DeArmond SJ, Stites DP, Prusiner SB. Antibodies to a scrapie

prion protein. Nature, Issue: 5976. 310: 418-21, 1984.

14. DeArmond SJ e, Eng LF. Immunohistochemistry: techniques and application to

neurooncology. Prog Exp Tumor Res, 27: 92-117, 1984.

15. Prusiner SB, Barry RA, McKinley MP, Bellinger CG, Meyer RK, DeArmond SJ, Kingsbury

DT. Scrapie and Creutzfeldt-Jakob disease prions. Microbiol Sci, Issue: 2. 2: 33-9, 1985.

16. Nagashima T, DeArmond SJ, Murovic J, Hoshino T. Immunocytochemical demonstration of

S-phase cells by anti-bromodeoxyuridine monoclonal antibody in human brain tumor tissues. Acta Neuropathol (Berl), Issue: 1-2. 67: 155-9, 1985.

17. DeArmond SJ, McKinley MP, Barry RA, Braunfeld MB, McColloch JR, Prusiner SB.

Identification of prion amyloid filaments in scrapie-infected brain. Cell, Issue: 1. 41: 221-35, May/1985.

18. Barry RA, McKinley MP, Bendheim PE, Lewis GK, DeArmond SJ, Prusiner SB. Antibodies

to the scrapie protein decorate prion rods. J Immunol, Issue: 1. 135: 603-13, Jul/1985.

19. Liu HC, DeArmond SJ, Edwards MS. An unusual spinal meningioma in a child: case report.

Neurosurgery, Issue: 2. 17: 313-6, Aug/1985.

20. Monteiro ML, Swanson RA, Coppeto JR, Cuneo RA, DeArmond SJ, Prusiner SB. A

microangiopathic syndrome of encephalopathy, hearing loss, and retinal arteriolar occlusions. Neurology, Issue: 8. 35: 1113-21, Aug/1985.

21. Kretzschmar HA, DeArmond SJ, Forno LS. Measurement of GFAP in hepatic

encephalopathy by ELISA and transblots. J Neuropathol Exp Neurol, Issue: 5. 44: 459-71, Sep/1985.

22. Koch TK, Berg BO, DeArmond SJ, Gravina RF. Creutzfeldt-Jakob disease in a young adult

with idiopathic hypopituitarism. Possible relation to the administration of cadaveric human growth hormone. N Engl J Med, Issue: 12. 313: 731-3, Sep/19/1985.

23. Kretzschmar HA, Prusiner SB, Stowring LE, DeArmond SJ. Scrapie prion proteins are

synthesized in neurons. Am J Pathol, Issue: 1. 122: 1-5, Jan/1986.

24. Hoshino T, Nagashima T, Murovic JA, Wilson CB, Edwards MS, Gutin PH, Davis RL,

DeArmond SJ. In situ cell kinetics studies on human neuroectodermal tumors with bromodeoxyuridine labeling. J Neurosurg, Issue: 3. 64: 453-9, Mar/1986.

25. Nagashima T, Murovic JA, Hoshino T, Wilson CB, DeArmond SJ. The proliferative potential

of human pituitary tumors in situ. J Neurosurg, Issue: 4. 64: 588-93, Apr/1986.


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26. Scharre D, Petri M, Engman E, DeArmond SJ. Large intracranial arteritis with giant cells in

systemic lupus erythematosus. Ann Intern Med, Issue: 5. 104: 661-2, May/1986.

27. Rutka JT, Giblin J, Dougherty DV, McCulloch JR, DeArmond SJ, Rosenblum ML. An

ultrastructural and immunocytochemical analysis of leptomeningeal and meningioma cultures. J Neuropathol Exp Neurol, Issue: 3. 45: 285-303, May/1986.

28. Barry RA, Kent SB, McKinley MP, Meyer RK, DeArmond SJ, Hood LE, Prusiner SB. Scrapie

and cellular prion proteins share polypeptide epitopes. J Infect Dis, Issue: 5. 153: 848-54, May/1986.

29. Rutka JT, Kleppe-Hoifodt H, Emma DA, Giblin JR, Dougherty DV, McCulloch JR,

DeArmond SJ, Rosenblum ML. Characterization of normal human brain cultures. Evidence for the outgrowth of leptomeningeal cells. Lab Invest, Issue: 1. 55: 71-85, Jul/1986.

30. Sze G, DeArmond SJ, Brant-Zawadzki M, Davis RL, Norman D, Newton TH. Foci of MRI

signal (pseudo lesions) anterior to the frontal horns: histologic correlations of a normal finding. AJR Am J Roentgenol, Issue: 2. 147: 331-7, Aug/1986.

31. Kretzschmar HA, Stowring LE, Westaway D, Stubblebine WH, Prusiner SB, Dearmond SJ.

Molecular cloning of a human prion protein cDNA. DNA, Issue: 4. 5: 315-24, Aug/1986.

32. Kitamoto T, Tateishi J, Tashima T, Takeshita I, Barry RA, DeArmond SJ, Prusiner SB.

Amyloid plaques in Creutzfeldt-Jakob disease stain with prion protein antibodies. Ann Neurol, Issue: 2. 20: 204-8, Aug/1986.

33. Carlson GA, Kingsbury DT, Goodman PA, Coleman S, Marshall ST, DeArmond S,

Westaway D, Prusiner SB. Linkage of prion protein and scrapie incubation time genes. Cell, Issue: 4. 46: 503-11, Aug/15/1986.

34. Murovic JA, DeArmond S, Nagashima T, Edwards MS, Hoshino T. Cell kinetics analysis in a

case of teratoma of the thoracic spine. J Neurosurg, Issue: 3. 65: 331-4, Sep/1986.

35. Chang KN, DeArmond SJ, Buncke HJ. Sensory reinnervation in microsurgical

reconstruction of the heel. Plast Reconstr Surg, Issue: 5. 78: 652-64, Nov/1986.

36. DeArmond SJ, Lee YL, Kretzschmar HA, Eng LF. Turnover of glial filaments in mouse

spinal cord. J Neurochem, Issue: 6. 47: 1749-53, Dec/1986.

37. Adams M, Rhyner PA, Day J, DeArmond S, Smuckler EA. Whipple's disease confined to the

central nervous system. Ann Neurol, Issue: 1. 21: 104-8, Jan/1987.

38. Kretzschmar HA, DeArmond SJ, Koch TK, Patel MS, Newth CJ, Schmidt KA, Packman S.

Pyruvate dehydrogenase complex deficiency as a cause of subacute necrotizing encephalopathy (Leigh disease). Pediatrics, Issue: 3. 79: 370-3, Mar/1987.

39. Prusiner SB, DeArmond SJ. Prions causing nervous system degeneration. Lab Invest,

Issue: 4. 56: 349-63, Apr/1987.


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40. Rutka JT, Giblin JR, Apodaca G, DeArmond SJ, Stern R, Rosenblum ML. Inhibition of

growth and induction of differentiation in a malignant human glioma cell line by normal leptomeningeal extracellular matrix proteins. Cancer Res, Issue: 13. 47: 3515-22, Jul/1/1987.

41. DeArmond SJ, Mobley WC, DeMott DL, Barry RA, Beckstead JH, Prusiner SB. Changes in

the localization of brain prion proteins during scrapie infection. Neurology, Issue: 8. 37: 1271-80, Aug/1987.

42. Prusiner SB, Stahl N, DeArmond SJ. Novel mechanisms of degeneration of the central

nervous system--prion structure and biology. Ciba Found Symp, 135: 239-60, 1988.

43. Germano IM, Pitts LH, Berry I, DeArmond SJ. High energy phosphate metabolism in

experimental permanent focal cerebral ischemia: an in vivo 31P magnetic resonance spectroscopy study. J Cereb Blood Flow Metab, Issue: 1. 8: 24-31, Feb/1988.

44. Gonzales MF, Olney RK, So YT, Greco CM, McQuinn BA, Miller RG, DeArmond SJ.

Subacute structural myopathy associated with human immunodeficiency virus infection. Arch Neurol, Issue: 5. 45: 585-7, May/1988.

45. Charman HP, Lowenstein DH, Cho KG, DeArmond SJ, Wilson CB. Primary cerebral

angiosarcoma. Case report. J Neurosurg, Issue: 5. 68: 806-10, May/1988.

46. Cho KG, DeArmond SJ, Barnwell S, Edwards MS, Hoshino T. Proliferative characteristics of

intracranial and spinal tumors of developmental origin. Cancer, Issue: 4. 62: 740-8, Aug/15/1988.

47. Rutka JT, DeArmond SJ, Giblin J, McCulloch JR, Wilson CB, Rosenblum ML. Effect of

retinoids on the proliferation, morphology and expression of glial fibrillary acidic protein of an anaplastic astrocytoma cell line. Int J Cancer, Issue: 3. 42: 419-27, Sep/15/1988.

48. Snow AD, Kisilevsky R, Willmer J, Prusiner SB, DeArmond SJ. Sulfated

glycosaminoglycans in amyloid plaques of prion diseases. Acta Neuropathol (Berl), Issue: 4. 77: 337-42, 1989.

49. DeArmond SJ, Gonzales M, Mobley WC, Kon AA, Stern A, Prusiner H, Prusiner SB. PrPSc

in scrapie-infected hamster brain is spatially and temporally related to histopathology and infectivity titer. Prog Clin Biol Res, 317: 601-18, 1989.

50. Gabizon R, McKinley MP, Groth D, Westaway D, DeArmond SJ, Carlson GA, Prusiner SB.

Immunoaffinity purification and neutralization of scrapie prions. Prog Clin Biol Res, 317: 583-600, 1989.

51. Rosenberg RN, Green JB, White CL, Sparkman DR, DeArmond SJ, Kepes JJ. Dominantly

inherited dementia and parkinsonism, with non-Alzheimer amyloid plaques: a new neurogenetic disorder. Ann Neurol, Issue: 2. 25: 152-8, Feb/1989.

52. Carlson GA, Westaway D, DeArmond SJ, Peterson-Torchia M, Prusiner SB. Primary

structure of prion protein may modify scrapie isolate properties. Proc Natl Acad Sci U S A, Issue: 19. 86: 7475-9, Oct/1989.

53. McKinley MP, DeArmond SJ, Torchia M, Mobley WC, Prusiner SB. Acceleration of scrapie

in neonatal Syrian hamsters. Neurology, Issue: 10. 39: 1319-24, Oct/1989.


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54. Rutka JT, Rosenblum ML, Stern R, Ralston HJ, Dougherty D, Giblin J, DeArmond S.

Isolation and partial purification of growth factors with TGF-like activity from human malignant gliomas. J Neurosurg, Issue: 6. 71: 875-83, Dec/1989.

55. Scott M, Foster D, Mirenda C, Serban D, Coufal F, Wälchli M, Torchia M, Groth D, Carlson

G, DeArmond SJ. Transgenic mice expressing hamster prion protein produce species-specific scrapie infectivity and amyloid plaques. Cell, Issue: 5. 59: 847-57, Dec/1/1989.

56. Serban D, Taraboulos A, DeArmond SJ, Prusiner SB. Rapid detection of Creutzfeldt-Jakob

disease and scrapie prion proteins. Neurology, Issue: 1. 40: 110-7, Jan/1990.

57. Prusiner SB, DeArmond SJ. Prion diseases of the central nervous system. Monogr Pathol,

Issue: 32. 86-122, 1990.

58. Lowenstein DH, Butler DA, Westaway D, McKinley MP, DeArmond SJ, Prusiner SB. Three

hamster species with different scrapie incubation times and neuropathological features encode distinct prion proteins. Mol Cell Biol, Issue: 3. 10: 1153-63, Mar/1990.

59. Boylan KB, Chin JH, DeArmond SJ. Progressive dystonia following resuscitation from

cardiac arrest. Neurology, Issue: 9. 40: 1458-61, Sep/1990.

60. Snow AD, Wight TN, Nochlin D, Koike Y, Kimata K, DeArmond SJ, Prusiner SB.

Immunolocalization of heparan sulfate proteoglycans to the prion protein amyloid plaques of Gerstmann-Straussler syndrome, Creutzfeldt-Jakob disease and scrapie. Lab Invest, Issue: 5. 63: 601-11, Nov/1990.

61. Hsiao KK, Scott M, Foster D, Groth DF, DeArmond SJ, Prusiner SB. Spontaneous

neurodegeneration in transgenic mice with mutant prion protein. Science, Issue: 4987. 250: 1587-90, Dec/14/1990.

62. Epstein CJ, Foster DB, DeArmond SJ, Prusiner SB. Acceleration of scrapie in trisomy 16----

diploid aggregation chimeras. Ann Neurol, Issue: 1. 29: 95-7, Jan/1991.

63. Holtzman DM, Li YW, Gage FH, DeArmond SJ, Epstein CJ, McKinley MP, Mobley WC.

Modeling cholinergic abnormalities in Down syndrome and Alzheimer's disease. Prog Clin Biol Res, 373: 189-202, 1991.

64. Hsiao K, Scott M, Foster D, DeArmond SJ, Groth D, Serban H, Prusiner SB. Spontaneous

neurodegeneration in transgenic mice with prion protein codon 101 proline----leucine substitution. Ann N Y Acad Sci, 640: 166-70, 1991.

65. Westaway D, Mirenda CA, Foster D, Zebarjadian Y, Scott M, Torchia M, Yang SL, Serban

H, DeArmond SJ, Ebeling C. Paradoxical shortening of scrapie incubation times by expression of prion protein transgenes derived from long incubation period mice. Neuron, Issue: 1. 7: 59-68, Jul/1991.

66. Prusiner SB, DeArmond SJ. Molecular biology and pathology of scrapie and the prion

diseases of humans. Brain Pathol, Issue: 4. 1: 297-310, Jul/1991.

67. Schendel SA, Pearl RM, DeArmond SJ. Pathophysiology of cleft lip muscles following the

initial surgical repair. Plast Reconstr Surg, Issue: 2. 88: 197-200, Aug/1991.


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68. Jendroska K, Heinzel FP, Torchia M, Stowring L, Kretzschmar HA, Kon A, Stern A, Prusiner

SB, DeArmond SJ. Proteinase-resistant prion protein accumulation in Syrian hamster brain correlates with regional pathology and scrapie infectivity. Neurology, Issue: 9. 41: 1482-90, Sep/1991.

69. McKinley MP, Taraboulos A, Kenaga L, Serban D, Stieber A, DeArmond SJ, Prusiner SB,

Gonatas N. Ultrastructural localization of scrapie prion proteins in cytoplasmic vesicles of infected cultured cells. Lab Invest, Issue: 6. 65: 622-30, Dec/1991.

70. DeArmond SJ, Kristensson K, Bowler RP. PrPSc causes nerve cell death and stimulates

astrocyte proliferation: a paradox. Prog Brain Res, 94: 437-46, 1992.

71. Holtzman DM, Li YW, DeArmond SJ, McKinley MP, Gage FH, Epstein CJ, Mobley WC.

Mouse model of neurodegeneration: atrophy of basal forebrain cholinergic neurons in trisomy 16 transplants. Proc Natl Acad Sci U S A, Issue: 4. 89: 1383-7, Feb/15/1992.

72. Kretzschmar HA, Kufer P, Riethmüller G, DeArmond S, Prusiner SB, Schiffer D. Prion

protein mutation at codon 102 in an Italian family with Gerstmann-Sträussler-Scheinker syndrome. Neurology, Issue: 4. 42: 809-10, Apr/1992.

73. Büeler H, Fischer M, Lang Y, Bluethmann H, Lipp HP, DeArmond SJ, Prusiner SB, Aguet

M, Weissmann C. Normal development and behaviour of mice lacking the neuronal cell-surface PrP protein. Nature, Issue: 6370. 356: 577-82, Apr/16/1992.

74. Hecker R, Taraboulos A, Scott M, Pan KM, Yang SL, Torchia M, Jendroska K, DeArmond

SJ, Prusiner SB. Replication of distinct scrapie prion isolates is region specific in brains of transgenic mice and hamsters. Genes Dev, Issue: 7. 6: 1213-28, Jul/1992.

75. Taraboulos A, Jendroska K, Serban D, Yang SL, DeArmond SJ, Prusiner SB. Regional

mapping of prion proteins in brain. Proc Natl Acad Sci U S A, Issue: 16. 89: 7620-4, Aug/15/1992.

76. Kaye BR, Neuwelt CM, London SS, DeArmond SJ. Central nervous system systemic lupus

erythematosus mimicking progressive multifocal leucoencephalopathy. Ann Rheum Dis, Issue: 10. 51: 1152-6, Oct/1992.

77. Scott M, Groth D, Foster D, Torchia M, Yang SL, DeArmond SJ, Prusiner SB. Propagation

of prions with artificial properties in transgenic mice expressing chimeric PrP genes. Cell, Issue: 5. 73: 979-88, Jun/4/1993.

78. DeArmond SJ, Yang SL, Lee A, Bowler R, Taraboulos A, Groth D, Prusiner SB. Three

scrapie prion isolates exhibit different accumulation patterns of the prion protein scrapie isoform. Proc Natl Acad Sci U S A, Issue: 14. 90: 6449-53, Jul/15/1993.

79. DeArmond SJ. Overview of the transmissible spongiform encephalopathies: prion protein

disorders. Br Med Bull, Issue: 4. 49: 725-37, Oct/1993.

80. Kristensson K, Feuerstein B, Taraboulos A, Hyun WC, Prusiner SB, DeArmond SJ. Scrapie

prions alter receptor-mediated calcium responses in cultured cells. Neurology, Issue: 11. 43: 2335-41, Nov/1993.


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81. DeArmond SJ, Prusiner SB. The neurochemistry of prion diseases. J Neurochem, Issue: 5.

61: 1589-601, Nov/1993.

82. Prusiner SB, Groth D, Serban A, Koehler R, Foster D, Torchia M, Burton D, Yang SL,

DeArmond SJ. Ablation of the prion protein (PrP) gene in mice prevents scrapie and facilitates production of anti-PrP antibodies. Proc Natl Acad Sci U S A, Issue: 22. 90: 10608-12, Nov/15/1993.

83. DeArmond SJ. Alzheimer's disease and Creutzfeldt-Jakob disease: overlap of pathogenic

mechanisms. Curr Opin Neurol, Issue: 6. 6: 872-81, Dec/1993.

84. DeArmond SJ, Stowring L, Amar A, Coopersmith P, Dougherty D, Spencer D, Mikkelsen T,

Rosenblum M. Development of a non-selecting, non-perturbing method to study human brain tumor cell invasion in murine brain. J Neurooncol, Issue: 1. 20: 27-34, 1994.

85. Prusiner SB, DeArmond SJ. Prion diseases and neurodegeneration. Annu Rev Neurosci,

17: 311-39, 1994.

86. Amar AP, DeArmond SJ, Spencer DR, Coopersmith PF, Ramos DM, Rosenblum ML.

Development of an in vitro extracellular matrix assay for studies of brain tumor cell invasion. J Neurooncol, Issue: 1. 20: 1-15, 1994.

87. Westaway D, DeArmond SJ, Cayetano-Canlas J, Groth D, Foster D, Yang SL, Torchia M,

Carlson GA, Prusiner SB. Degeneration of skeletal muscle, peripheral nerves, and the central nervous system in transgenic mice overexpressing wild-type prion proteins. Cell, Issue: 1. 76: 117-29, Jan/14/1994.

88. DeArmond SJ, Yang SL, Cayetano-Canlas J, Groth D, Prusiner SB. The neuropathological

phenotype in transgenic mice expressing different prion protein constructs. Philos Trans R Soc Lond B Biol Sci, Issue: 1306. 343: 415-23, Mar/29/1994.

89. Carlson GA, DeArmond SJ, Torchia M, Westaway D, Prusiner SB. Genetics of prion

diseases and prion diversity in mice. Philos Trans R Soc Lond B Biol Sci, Issue: 1306. 343: 363-9, Mar/29/1994.

90. Lane KL, Brown P, Howell DN, Crain BJ, Hulette CM, Burger PC, DeArmond SJ.

Creutzfeldt-Jakob disease in a pregnant woman with an implanted dura mater graft. Neurosurgery, Issue: 4. 34: 737-9; discussion 739-40, Apr/1994.

91. Carlson GA, Ebeling C, Yang SL, Telling G, Torchia M, Groth D, Westaway D, DeArmond

SJ, Prusiner SB. Prion isolate specified allotypic interactions between the cellular and scrapie prion proteins in congenic and transgenic mice. Proc Natl Acad Sci U S A, Issue: 12. 91: 5690-4, Jun/7/1994.

92. Hsiao KK, Groth D, Scott M, Yang SL, Serban H, Rapp D, Foster D, Torchia M, DeArmond

SJ, Prusiner SB. Serial transmission in rodents of neurodegeneration from transgenic mice expressing mutant prion protein. Proc Natl Acad Sci U S A, Issue: 19. 91: 9126-30, Sep/13/1994.

93. Telling GC, Scott M, Hsiao KK, Foster D, Yang SL, Torchia M, Sidle KC, Collinge J,

DeArmond SJ, Prusiner SB. Transmission of Creutzfeldt-Jakob disease from humans to


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transgenic mice expressing chimeric human-mouse prion protein. Proc Natl Acad Sci U S A, Issue: 21. 91: 9936-40, Oct/11/1994.

94. Jendroska K, Poewe W, Daniel SE, Pluess J, Iwerssen-Schmidt H, Paulsen J, Barthel S,

Schelosky L, Cervós-Navarro J, DeArmond SJ. Ischemic stress induces deposition of amyloid beta immunoreactivity in human brain. Acta Neuropathol (Berl), Issue: 5. 90: 461-6, 1995.

95. DeArmond SJ, Prusiner SB. Prion protein transgenes and the neuropathology in prion

diseases. Brain Pathol, Issue: 1. 5: 77-89, Jan/1995.

96. DeArmond SJ, Prusiner SB. Etiology and pathogenesis of prion diseases. Am J Pathol,

Issue: 4. 146: 785-811, Apr/1995.

97. Telling GC, Scott M, Mastrianni J, Gabizon R, Torchia M, Cohen FE, DeArmond SJ,

Prusiner SB. Prion propagation in mice expressing human and chimeric PrP transgenes implicates the interaction of cellular PrP with another protein. Cell, Issue: 1. 83: 79-90, Oct/6/1995.

98. Mastrianni JA, Curtis MT, Oberholtzer JC, Da Costa MM, DeArmond SJ, Prusiner SB,

Garbern JY. Prion disease (PrP-A117V) presenting with ataxia instead of dementia. Neurology, Issue: 11. 45: 2042-50, Nov/1995.

99. Wille H, Baldwin MA, Cohen FE, DeArmond SJ, Prusiner SB. Prion protein amyloid:

separation of scrapie infectivity from PrP polymers. Ciba Found Symp, 199: 181-99; discussion 199-201, 1996.

100. DeArmond SJ, Qiu Y, Wong K, Nixon R, Hyun W, Prusiner SB, Mobley WC. Abnormal

plasma membrane properties and functions in prion-infected cell lines. Cold Spring Harb Symp Quant Biol, 61: 531-40, 1996.

101. DeArmond SJ, Prusiner SB. Transgenetics and neuropathology of prion diseases. Curr Top

Microbiol Immunol, 207: 125-46, 1996.

102. Lledo PM, Tremblay P, DeArmond SJ, Prusiner SB, Nicoll RA. Mice deficient for prion

protein exhibit normal neuronal excitability and synaptic transmission in the hippocampus. Proc Natl Acad Sci U S A, Issue: 6. 93: 2403-7, Mar/19/1996.

103. Williamson RA, Peretz D, Smorodinsky N, Bastidas R, Serban H, Mehlhorn I, DeArmond

SJ, Prusiner SB, Burton DR. Circumventing tolerance to generate autologous monoclonal antibodies to the prion protein. Proc Natl Acad Sci U S A, Issue: 14. 93: 7279-82, Jul/9/1996.

104. Telling GC, Haga T, Torchia M, Tremblay P, DeArmond SJ, Prusiner SB. Interactions

between wild-type and mutant prion proteins modulate neurodegeneration in transgenic mice. Genes Dev, Issue: 14. 10: 1736-50, Jul/15/1996.

105. Tatzelt J, Maeda N, Pekny M, Yang SL, Betsholtz C, Eliasson C, Cayetano J, Camerino AP,

DeArmond SJ, Prusiner SB. Scrapie in mice deficient in apolipoprotein E or glial fibrillary acidic protein. Neurology, Issue: 2. 47: 449-53, Aug/1996.


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106. Diez M, Koistinaho J, DeArmond SJ, Camerino AP, Groth D, Caytano JC, Prusiner SB,

Hökfelt T. Aberrant induction of neuropeptide Y mRNA in hippocampal CA3 pyramidal neurones in scrapie-infected mice. Neuroreport, Issue: 12. 7: 1887-92, Aug/12/1996.

107. Kretzschmar HA, Ironside JW, DeArmond SJ, Tateishi J. Diagnostic criteria for sporadic

Creutzfeldt-Jakob disease. Arch Neurol, Issue: 9. 53: 913-20, Sep/1996.

108. Wong K, Qiu Y, Hyun W, Nixon R, VanCleff J, Sanchez-Salazar J, Prusiner SB, DeArmond

SJ. Decreased receptor-mediated calcium response in prion-infected cells correlates with decreased membrane fluidity and IP3 release. Neurology, Issue: 3. 47: 741-50, Sep/1996.

109. Mastrianni JA, Iannicola C, Myers RM, DeArmond SJ, Prusiner SB. Mutation of the prion

protein gene at codon 208 in familial Creutzfeldt-Jakob disease. Neurology, Issue: 5. 47: 1305-12, Nov/1996.

110. Vey M, Pilkuhn S, Wille H, Nixon R, DeArmond SJ, Smart EJ, Anderson RG, Taraboulos A,

Prusiner SB. Subcellular colocalization of the cellular and scrapie prion proteins in caveolae-like membranous domains. Proc Natl Acad Sci U S A, Issue: 25. 93: 14945-9, Dec/10/1996.

111. Telling GC, Parchi P, DeArmond SJ, Cortelli P, Montagna P, Gabizon R, Mastrianni J,

Lugaresi E, Gambetti P, Prusiner SB. Evidence for the conformation of the pathologic isoform of the prion protein enciphering and propagating prion diversity. Science, Issue: 5295. 274: 2079-82, Dec/20/1996.

112. Carlson GA, Banks S, Lund D, Reichert C, Groth D, Torchia M, DeArmond SJ, Prusiner SB.

Failure to transmit disease from gray tremor mutant mice. J Virol, Issue: 3. 71: 2342-5, Mar/1997.

113. Telling GC, Tremblay P, Torchia M, DeArmond SJ, Cohen FE, Prusiner SB. N-terminally

tagged prion protein supports prion propagation in transgenic mice. Protein Sci, Issue: 4. 6: 825-33, Apr/1997.

114. Muramoto T, DeArmond SJ, Scott M, Telling GC, Cohen FE, Prusiner SB. Heritable

disorder resembling neuronal storage disease in mice expressing prion protein with deletion of an alpha-helix. Nat Med, Issue: 7. 3: 750-5, Jul/1997.

115. MacGowan DJ, Delanty N, Petito F, Edgar M, Mastrianni J, DeArmond SJ. Isolated

myoclonic alien hand as the sole presentation of pathologically established Creutzfeldt-Jakob disease: a report of two patients. J Neurol Neurosurg Psychiatry, Issue: 3. 63: 404-7, Sep/1997.

116. Schätzl HM, Laszlo L, Holtzman DM, Tatzelt J, DeArmond SJ, Weiner RI, Mobley WC,

Prusiner SB. A hypothalamic neuronal cell line persistently infected with scrapie prions exhibits apoptosis. J Virol, Issue: 11. 71: 8821-31, Nov/1997.

117. Diez M, Koistinaho J, Dearmond SJ, Groth D, Prusiner SB, Hökfelt T. Marked decrease of

neuropeptide Y Y2 receptor binding sites in the hippocampus in murine prion disease. Proc Natl Acad Sci U S A, Issue: 24. 94: 13267-72, Nov/25/1997.

118. DeArmond SJ, Sánchez H, Yehiely F, Qiu Y, Ninchak-Casey A, Daggett V, Camerino AP,

Cayetano J, Rogers M, Groth D, Torchia M, Tremblay P, Scott MR, Cohen FE, Prusiner SB. Selective neuronal targeting in prion disease. Neuron, Issue: 6. 19: 1337-48, Dec/1997.


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119. Scott MR, Groth D, Tatzelt J, Torchia M, Tremblay P, DeArmond SJ, Prusiner SB.

Propagation of prion strains through specific conformers of the prion protein. J Virol, Issue: 12. 71: 9032-44, Dec/1997.

120. Scott MR, Safar J, Telling G, Nguyen O, Groth D, Torchia M, Koehler R, Tremblay P,

Walther D, Cohen FE, DeArmond SJ, Prusiner SB. Identification of a prion protein epitope modulating transmission of bovine spongiform encephalopathy prions to transgenic mice. Proc Natl Acad Sci U S A, Issue: 26. 94: 14279-84, Dec/23/1997.

121. DeArmond SJ, Mobley WC, DeMott DL, Barry RA, Beckstead JH, Prusiner SB. Changes in

the localization of brain prion proteins during scrapie infection. Neurology, Issue: 1. 50: 1271-80; discussion 2, Jan/1998.

122. Hegde RS, Mastrianni JA, Scott MR, DeFea KA, Tremblay P, Torchia M, DeArmond SJ,

Prusiner SB, Lingappa VR. A transmembrane form of the prion protein in neurodegenerative disease. Science, Issue: 5352. 279: 827-34, Feb/6/1998.

123. Hökfelt T, Broberger C, Zhang X, Diez M, Kopp J, Xu Z, Landry M, Bao L, Schalling M,

Koistinaho J, DeArmond SJ, Prusiner S, Gong J, Walsh JH. Neuropeptide Y: some viewpoints on a multifaceted peptide in the normal and diseased nervous system. Brain Res Brain Res Rev, Issue: 2-3. 26: 154-66, May/1998.

124. Prusiner SB, Scott MR, DeArmond SJ, Cohen FE. Prion protein biology. Cell, Issue: 3. 93:

337-48, May/1/1998.

125. Evatt B, Austin H, Barnhart E, Schonberger L, Sharer L, Jones R, DeArmond SJ.

Surveillance for Creutzfeldt-Jakob disease among persons with hemophilia. Transfusion, Issue: 9. 38: 817-20, Sep/1998.

126. Tremblay P, Meiner Z, Galou M, Heinrich C, Petromilli C, Lisse T, Cayetano J, Torchia M,

Mobley W, Bujard H, DeArmond SJ, Prusiner SB. Doxycycline control of prion protein transgene expression modulates prion disease in mice. Proc Natl Acad Sci U S A, Issue: 21. 95: 12580-5, Oct/13/1998.

127. Phatouros CC, Higashida RT, Malek AM, Smith WS, Mully TW, DeArmond SJ, Dowd CF,

Halbach VV. Endovascular stenting of an acutely thrombosed basilar artery: technical case report and review of the literature. Neurosurgery, Issue: 3. 44: 667-73, Mar/1999.

128. Supattapone S, Bosque P, Muramoto T, Wille H, Aagaard C, Peretz D, Nguyen HO,

Heinrich C, Torchia M, Safar J, Cohen FE, DeArmond SJ, Prusiner SB, Scott M. Prion protein of 106 residues creates an artifical transmission barrier for prion replication in transgenic mice. Cell, Issue: 6. 96: 869-78, Mar/19/1999.

129. Mastrianni JA, Nixon R, Layzer R, Telling GC, Han D, DeArmond SJ, Prusiner SB. Prion

protein conformation in a patient with sporadic fatal insomnia. N Engl J Med, Issue: 21. 340: 1630-8, May/27/1999.

130. Nishida N, Tremblay P, Sugimoto T, Shigematsu K, Shirabe S, Petromilli C, Erpel SP,

Nakaoke R, Atarashi R, Houtani T, Torchia M, Sakaguchi S, DeArmond SJ, Prusiner SB, Katamine S. A mouse prion protein transgene rescues mice deficient for the prion protein gene from purkinje cell degeneration and demyelination. Lab Invest, Issue: 6. 79: 689-97, Jun/1999.


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131. Lomen-Hoerth C, Simmons ML, Dearmond SJ, Layzer RB. Adult-onset nemaline myopathy:

Another cause of dropped head. Muscle Nerve, Issue: 8. 22: 1146-50, Aug/1999.

132. DeArmond SJ, Qiu Y, Sànchez H, Spilman PR, Ninchak-Casey A, Alonso D, Daggett V.

PrPC glycoform heterogeneity as a function of brain region: implications for selective targeting of neurons by prion strains. J Neuropathol Exp Neurol, Issue: 9. 58: 1000-9, Sep/1999.

133. Tatzelt J, Groth DF, Torchia M, Prusiner SB, DeArmond SJ. Kinetics of prion protein

accumulation in the CNS of mice with experimental scrapie. J Neuropathol Exp Neurol, Issue: 12. 58: 1244-9, Dec/1999.

134. Hegde RS, Tremblay P, Groth D, DeArmond SJ, Prusiner SB, Lingappa VR. Transmissible

and genetic prion diseases share a common pathway of neurodegeneration. Nature, Issue: 6763. 402: 822-6, Dec/16/1999.

135. Huang CS, Zhou J, Feng AK, Lynch CC, Klumperman J, DeArmond SJ, Mobley WC. Nerve

growth factor signaling in caveolae-like domains at the plasma membrane. J Biol Chem, Issue: 51. 274: 36707-14, Dec/17/1999.

136. Scott MR, Will R, Ironside J, Nguyen HO, Tremblay P, DeArmond SJ, Prusiner SB.

Compelling transgenetic evidence for transmission of bovine spongiform encephalopathy prions to humans. Proc Natl Acad Sci U S A, Issue: 26. 96: 15137-42, Dec/21/1999.

137. Scott MR, Supattapone S, Nguyen HO, DeArmond SJ, Prusiner SB. Transgenic models of

prion disease. Arch Virol Suppl, Issue: 16. 113-24, 2000.

138. Kaneko K, Ball HL, Wille H, Zhang H, Groth D, Torchia M, Tremblay P, Safar J, Prusiner

SB, DeArmond SJ, Baldwin MA, Cohen FE. A synthetic peptide initiates Gerstmann-Sträussler-Scheinker (GSS) disease in transgenic mice. J Mol Biol, Issue: 4. 295: 997-1007, Jan/28/2000.

139. DeArmond SJ. Cerebral amyloidosis in prion diseases. Amyloid: Int. J. Exp. Clin. Invest.,

Issue: 1. 7: 3-6, Mar/2000.

140. Bouzamondo E, Milroy AM, Ralston HJ, Prusiner SB, DeArmond SJ. Selective neuronal

vulnerability during experimental scrapie infection: insights from an ultrastructural investigation. Brain Res, Issue: 2. 874: 210-5, Aug/25/2000.

141. Stephenson DA, Chiotti K, Ebeling C, Groth D, DeArmond SJ, Prusiner SB, Carlson GA.

Quantitative trait loci affecting prion incubation time in mice. Genomics, Issue: 1. 69: 47-53, Oct/1/2000.

142. Supattapone S, Nguyen HO, Muramoto T, Cohen FE, DeArmond SJ, Prusiner SB, Scott M.

Affinity-tagged miniprion derivatives spontaneously adopt protease-resistant conformations. J Virol, Issue: 24. 74: 11928-34, Dec/2000.

143. Supattapone S, Muramoto T, Legname G, Mehlhorn I, Cohen FE, DeArmond SJ, Prusiner

SB, Scott MR. Identification of two prion protein regions that modify scrapie incubation time. J Virol, Issue: 3. 75: 1408-13, Feb/2001.


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144. Alonso DO, DeArmond SJ, Cohen FE, Daggett V. Mapping the early steps in the pH-

induced conformational conversion of the prion protein. Proc Natl Acad Sci U S A, Issue: 6. 98: 2985-9, Mar/13/2001.

145. Supattapone S, Bouzamondo E, Ball HL, Wille H, Nguyen HO, Cohen FE, DeArmond SJ,

Prusiner SB, Scott M. A protease-resistant 61-residue prion peptide causes neurodegeneration in transgenic mice. Mol Cell Biol, Issue: 7. 21: 2608-16, Apr/2001.

146. Diez M, DeArmond SJ, Groth D, Prusiner SB, Hökfelt T. Decreased MK-801 binding in

discrete hippocampal regions of prion-infected mice. Neurobiol Dis, Issue: 4. 8: 692-9, Aug/2001.

147. Schmitt-Ulms G, Legname G, Baldwin MA, Ball HL, Bradon N, Bosque PJ, Crossin KL,

Edelman GM, DeArmond SJ, Cohen FE, Prusiner SB. Binding of neural cell adhesion molecules (N-CAMs) to the cellular prion protein. J Mol Biol, Issue: 5. 314: 1209-25, Dec/14/2001.

148. Moore RC, Mastrangelo P, Bouzamondo E, Heinrich C, Legname G, Prusiner SB, Hood L,

Westaway D, DeArmond SJ, Tremblay P. Doppel-induced cerebellar degeneration in transgenic mice. Proc Natl Acad Sci U S A, Issue: 26. 98: 15288-93, Dec/18/2001.

149. Mastrianni JA, Capellari S, Telling GC, Han D, Bosque P, Prusiner SB, DeArmond SJ.

Inherited prion disease caused by the V210I mutation: transmission to transgenic mice. Neurology, Issue: 12. 57: 2198-205, Dec/26/2001.

150. Bosque PJ, Ryou C, Telling G, Peretz D, Legname G, DeArmond SJ, Prusiner SB. Prions in

skeletal muscle. Proc Natl Acad Sci U S A, Issue: 6. 99: 3812-7, Mar/19/2002.

151. Peretz D, Williamson RA, Legname G, Matsunaga Y, Vergara J, Burton DR, DeArmond SJ,

Prusiner SB, Scott MR. A change in the conformation of prions accompanies the emergence of a new prion strain. Neuron, Issue: 6. 34: 921-32, Jun/13/2002.

152. Perrier V, Kaneko K, Safar J, Vergara J, Tremblay P, DeArmond SJ, Cohen FE, Prusiner

SB, Wallace AC. Dominant-negative inhibition of prion replication in transgenic mice. Proc Natl Acad Sci U S A, Issue: 20. 99: 13079-84, Oct/1/2002.

153. Legname G, Nelken P, Guan Z, Kanyo ZF, DeArmond SJ, Prusiner SB. Prion and doppel

proteins bind to granule cells of the cerebellum. Proc Natl Acad Sci U S A, Issue: 25. 99: 16285-90, Dec/10/2002.

154. DeArmond SJ, Bouzamondo E. Fundamentals of prion biology and diseases. Toxicology,

181-182: 9-16, Dec/27/2002.

155. Lewicki H, Tishon A, Homann D, Mazarguil H, Laval F, Asensio VC, Campbell IL,

DeArmond SJ, Coon B, Teng C, Gairin JE, Oldstone MB. T cells infiltrate the brain in murine and human transmissible spongiform encephalopathies. J Virol, Issue: 6. 77: 3799-808, Mar/2003.

156. DeArmond SJ, Prusiner SB. Perspectives on prion biology, prion disease pathogenesis, and

pharmacologic approaches to treatment. Clin Lab Med, Issue: 1. 23: 1-41, Mar/2003.

157. Korth C, Kaneko K, Groth D, Heye N, Telling G, Mastrianni J, Parchi P, Gambetti P, Will R,

Ironside J, Heinrich C, Tremblay P, DeArmond SJ, Prusiner SB. Abbreviated incubation times for


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human prions in mice expressing a chimeric mouse-human prion protein transgene. Proc Natl Acad Sci U S A, Issue: 8. 100: 4784-9, Apr/15/2003.

158. Martindale J, Geschwind MD, DeArmond SJ, Young G, Dillon WP, Henry R, Uyehara-Lock

JH, Gaskin DA, Miller BL. Sporadic Creutzfeldt-Jakob disease mimicking variant Creutzfeldt-Jakob disease. Arch Neurol, Issue: 5. 60: 767-70, May/2003.

159. Geschwind MD, Martindale J, Miller D, DeArmond SJ, Uyehara-Lock J, Gaskin D, Kramer

JH, Barbaro NM, Miller BL. Challenging the clinical utility of the 14-3-3 protein for the diagnosis of sporadic Creutzfeldt-Jakob disease. Arch Neurol, Issue: 6. 60: 813-6, Jun/2003.

160. Mironov A, Latawiec D, Wille H, Bouzamondo-Bernstein E, Legname G, Williamson RA,

Burton D, DeArmond SJ, Prusiner SB, Peters PJ. Cytosolic prion protein in neurons. J Neurosci, Issue: 18. 23: 7183-93, Aug/6/2003.

161. Peters PJ, Mironov A, Peretz D, van Donselaar E, Leclerc E, Erpel S, DeArmond SJ, Burton

DR, Williamson RA, Vey M, Prusiner SB. Trafficking of prion proteins through a caveolae-mediated endosomal pathway. J Cell Biol, Issue: 4. 162: 703-17, Aug/18/2003.

162. Tremblay P, Ball HL, Kaneko K, Groth D, Hegde RS, Cohen FE, DeArmond SJ, Prusiner

SB, Safar JG. Mutant PrPSc conformers induced by a synthetic peptide and several prion strains. J Virol, Issue: 4. 78: 2088-99, Feb/2004.

163. Schmitt-Ulms G, Hansen K, Liu J, Cowdrey C, Yang J, DeArmond SJ, Cohen FE, Prusiner

SB, Baldwin MA. Time-controlled transcardiac perfusion cross-linking for the study of protein interactions in complex tissues. Nat Biotechnol, Issue: 6. 22: 724-31, Jun/2004.

164. Legname G, Baskakov IV, Nguyen HO, Riesner D, Cohen FE, DeArmond SJ, Prusiner SB.

Synthetic mammalian prions. Science, Issue: 5684. 305: 673-6, Jul/30/2004.

165. Bouzamondo-Bernstein E, Hopkins SD, Spilman P, Uyehara-Lock J, Deering C, Safar J,

Prusiner SB, Ralston HJ, DeArmond SJ. The neurodegeneration sequence in prion diseases: evidence from functional, morphological and ultrastructural studies of the GABAergic system. J Neuropathol Exp Neurol, Issue: 8. 63: 882-99, Aug/2004.

166. Smith WS, Dowd CF, Johnston SC, Ko NU, DeArmond SJ, Dillon WP, Setty D, Lawton MT,

Young WL, Higashida RT, Halbach VV. Neurotoxicity of intra-arterial papaverine preserved with chlorobutanol used for the treatment of cerebral vasospasm after aneurysmal subarachnoid hemorrhage. Stroke, Issue: 11. 35: 2518-22, Nov/2004.

167. DeArmond SJ. Discovering the mechanisms of neurodegeneration in prion diseases.

Neurochem Res, Issue: 11. 29: 1979-98, Nov/2004.

168. Ishikura N, Clever JL, Bouzamondo-Bernstein E, Samayoa E, Prusiner SB, Huang EJ,

DeArmond SJ. Notch-1 activation and dendritic atrophy in prion disease. Proc Natl Acad Sci U S A, Issue: 3. 102: 886-91, Jan/18/2005.

169. Legname G, Nguyen HO, Baskakov IV, Cohen FE, DeArmond SJ, Prusiner SB. Strain-

specified characteristics of mouse synthetic prions. Proc Natl Acad Sci U S A, Issue: 6. 102: 2168-73, Feb/8/2005.


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170. Ashwath ML, DeArmond SJ, Culclasure T. Prion-associated dilated cardiomyopathy. Arch

Intern Med, Issue: 3. 165: 338-40, Feb/14/2005.

171. Safar JG, Geschwind MD, Deering C, Didorenko S, Sattavat M, Sanchez H, Serban A, Vey

M, Baron H, Giles K, Miller BL, DeArmond SJ, Prusiner SB. Diagnosis of human prion disease. Proc Natl Acad Sci U S A, Issue: 9. 102: 3501-6, Mar/1/2005.

172. Keet CA, Fox CK, Margeta M, Marco E, Shane AL, DeArmond SJ, Strober JB, Miller SP.

Infant botulism, type F, presenting at 54 hours of life. Pediatr Neurol, Issue: 3. 32: 193-6, Mar/2005.

173. Scott MR, Peretz D, Nguyen HO, DeArmond SJ, Prusiner SB. Transmission barriers for

bovine, ovine, and human prions in transgenic mice. J Virol, Issue: 9. 79: 5259-71, May/2005.

174. Safar JG, DeArmond SJ, Kociuba K, Deering C, Didorenko S, Bouzamondo-Bernstein E,

Prusiner SB, Tremblay P. Prion clearance in bigenic mice. J Gen Virol, Issue: Pt 10. 86: 2913-23, Oct/2005.

175. Enns GM, Hoppel CL, DeArmond SJ, Schelley S, Bass N, Weisiger K, Horoupian D,

Packman S. Relationship of primary mitochondrial respiratory chain dysfunction to fiber type abnormalities in skeletal muscle. Clin Genet, Issue: 4. 68: 337-48, Oct/2005.

176. Peretz D, Supattapone S, Giles K, Vergara J, Freyman Y, Lessard P, Safar JG, Glidden DV,

McCulloch C, Nguyen HO, Scott M, DeArmond SJ, Prusiner SB. Inactivation of prions by acidic sodium dodecyl sulfate. J Virol, Issue: 1. 80: 322-31, Jan/2006.

177. Rabinovici GD, Wang PN, Levin J, Cook L, Pravdin M, Davis J, DeArmond SJ, Barbaro NM,

Martindale J, Miller BL, Geschwind MD. First symptom in sporadic Creutzfeldt-Jakob disease. Neurology, Issue: 2. 66: 286-7, Jan/24/2006.

178. Lewis AM, Yu M, DeArmond SJ, Dillon WP, Miller BL, Geschwind MD. Human growth

hormone-related iatrogenic Creutzfeldt-Jakob disease with abnormal imaging. Arch Neurol, Issue: 2. 63: 288-90, Feb/2006.

179. Giri RK, Young R, Pitstick R, DeArmond SJ, Prusiner SB, Carlson GA. Prion infection of

mouse neurospheres. Proc Natl Acad Sci U S A, Feb/22/2006.

180. Diez M, Groth D, DeArmond SJ, Prusiner SB, Hökfelt T. Changes in neuropeptide

expression in mice infected with prions. Neurobiol Aging, Apr/16/2006.

181. Safar JG, Wille H, Geschwind MD, Deering C, Latawiec D, Serban A, King DJ, Legname G,

Weisgraber KH, Mahley RW, Miller BL, DeArmond SJ, Prusiner SB. Human prions and plasma lipoproteins. Proc Natl Acad Sci U S A, Issue: 30. 103: 11312-7, Jul/25/2006.

182. Tamgüney G, Giles K, Bouzamondo-Bernstein E, Bosque PJ, Miller MW, Safar J,

DeArmond SJ, Prusiner SB. Transmission of elk and deer prions to transgenic mice. J Virol, Issue: 18. 80: 9104-14, 2006.

183. Seeley WW, Carlin DA, Allman JM, Macedo MN, Bush C, Miller BL, DeArmond SJ. Early

frontotemporal dementia targets neurons unique to apes and humans. Ann Neurol 60: 660-7, 2006.


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184. Legname G, Nguyen HO, Peretz D, Cohen FE, DeArmond SJ, Prusiner SB. Continuum of

prion protein structures enciphers a multitude of prion isolate-specified phenotypes. Proc Natl Acad Sci U S A, Issue: 50. 103: 19105-10, 2006.

185. Tremblay P, Bouzamondo-Bernstein E, Heinrich C, Prusiner SB, DeArmond, SJ.

Developmental expression of PrP in the post-implantation embryo. Brain Research 1139:60-67, 2007.

186. Diez M, Groth D, DeArmond SJ, Prusiner SB, Hökfelt. Changes in neuropeptide expression

in mice infected with prions. Neurobiology of Aging 28:748-765, 2007.

187. Kim EJ, Rabinovici GD, Seeley WW, Halabi C, Shu H, Weiner MW, DeArmond SJ,

Trojanowski JQ, Gorno-Tempini ML, Miller BL, Rosen HJ. Patterns of MRI atrophy in tau-positive and ubiquitin-positive frontotemporal lobar degeneration. J Neurol Neurosurg Psychiatry 78:1375-8, 2007.

188. Seeley WW, Allman JM, Carlin DA, Crawford RK, Macedo MN, Greicius MD, DeArmond SJ,

Miller BL. Divergent social functioning in behavioral variant frontotemporal dementia and Alzheimer disease: reciprocal networks and neuronal evolution. Alzheimer Dis Assoc Disord. 21(4):S50-7, 2007.

189. Rabinovici GD, Seeley WW, Kim EJ, Gorno-Tempini ML, Rascovsky K, Pagliaro TA, Allison

SC, Halabi C, Kramer JH, Johnson HK, Weiner MW, Forman MS, Trojanowski JQ, DeArmond SJ, Miller BL, Rosen HJ. Distinct MRI athropy patterns in autopsy-proven Alzheimer's disease and frontotemporal lobar degenration. Am J Alzheimers Disease and Other Dementias 22: Issue 6, 474-88, 2007.

190. Safar JG, Lessard P, Tamgüney G, Freyman Y, Deering C, Letessier F, DeArmond SJ,

Prusiner SB. Transmission and detection of prions in feces. Journal of Infectious Diseases 197: Issue 1, 81-9, 2008.

191. Spilman P, Lessard P, Sattavat M, Bush C, Tousseyn T, Huang EJ, Giles K, Golde T, Das

P, Fauq A, Prusiner SB, DeArmond SJ. A gamma-secretase inhibitor and quinacrine reduce prions and prevent dendritic degeneration in murine brains. Proc Natl Acad Sci U S A: 10595-10600, 2008.

192. Tunnell E, Wollman R, Mallik S, Cortes CJ, DeArmond SJ, Mastrianni JA. A novel PRNP-

P105S mutation associated with atypical prion disease and a rare PrPSc conformation. Neruology 71(18): 1431-8. 2008

193. Giles K, Glidden DV, Beckwith R, Seoanes R, Peretz D, DeArmond SJ, Prusiner SB.

Resistance of bovine spongiform encephalopathy (BSE) prions to inactivation. PLoS Pathog. 4(11):e1000206, 2008.

194. Godsave SF, Wille H, Kujala P, Latawiec D, DeArmond SJ, Serban A, Prusiner SB, Peters

PJ. Cryo-immunogold electron microscopy for prions: toward identification of a conversion site. J Neurosci. 28(47):12489-99, 2008

195. Tamguney G, Miller MW, Giles K, Lemus A, Glidden DV, DeArmond SJ, Prusiner SB.

Transmission of scrapie and sheep-passaged bovine spongiform encephalopathy prions to transgenic mice expressing elk prion protein. J Gen Virol. 90(Pt4):1035-47, 2009.


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196. Geschwind MD, Potter CA, Sattavat M, Garcia PA, Rosen HJ, Miller BL, DeArmond SJ.

Correlating DWI MRI with pathological and other features of Creutzfeldt-Jakob disease. Alzheimer's Disease and Associated Disorders 23: Issue 1, 82-87, 2009.

197. Hwang D, Lee IY, Yoo H, Gehlenborg N, Cho JH, Petritis B, Baxter D, Pitstick R, Young R,

Spicer D, Price ND, Hohmann JG, DeArmond SJ, Carlson GA, Hood LE. A systems approach to prion disease. Mol Syst Biol. 5:252, 2009.

198. Tamgüney G, Francis KP, Giles K, Lemus A, DeArmond SJ, Prusiner SB. Measuring prions

by bioluminescence imaging. Proc Natl Acad Sci USA. 2009 (Epub ahead of print).

199. Yang W, Cook J, Rassbach B, Lemus A, DeArmond SJ, Mastrianni JA. A new transgenic

mouse model of Gerstmann-Straussler-Scheinker syndrome cause by the A117V mutation of PRNP. J Neurosci. 29(32):10072-80, 2009.

200. Douglas VC, DeArmond SJ, Aminoff MJ, Miller BL, Rabinovici GD. Seizures in corticobasal

degeneration: A case report. Neurocase. 18:1-5, 2009.

201. Irodenko VS, Lee HS, DeArmond SJ, Layzer RB. Adult nemaline myopathy with trabecular

muscle fibers. Muscle Nerve. 39(6):871-5, 2009.

202. Tamgüney G, Miller MW, Wofe LL, Sirochman TM, Glidden DV, Palmer C, Lemus A,

DeArmond SJ, Prusiner SB. Asymptomatic deer excrete infections prions in faeces. Nature. 2009. (Epub ahead of print).

203. Tartaglia MC, Sidhu M, Laluz V, Racine C, Rabinovici GD, Creighton K, Karydas A,

Rademakers R, Huang EJ, Miller BL, DeArmond SJ, Seeley WW. Sporadic corticobasal syndrome due to FTLD-TDP. Acta Neuropathol. 119(3):365-374 2010. (Epub Oct 2009). PMID: 19876635

204. Colby DW, Giles K, Legname G, Wille H, Baskakov IV, DeArmond SJ, Prusiner SB. Design

and construction of diverse mammalian prion strains. Proc Natl Acad Sci USA. 2009 Dec 1;106(48):20417-22. Epub 2009 Nov 13. PMID: 19915150

205. Ghaemmaghami S, Ahn M, Lessard P, Giles K, Legname G, DeArmond SJ, Prusiner SB.

Continuous quinacrine treatment results in the formation of drug-resistant prions. PLoS Pathog. 2009 Nov;5(11):e1000673. Epub 2009 Nov 26. PMID: 19956709

206. Colby DW, Wain R, Baskakov IV, Legname G, Palmer CG, Nguyen HO, Lemus A, Cohen

FE, DeArmond SJ, Prusiner SB. Protease-sensitive synthetic prions. PLoS Pathog. 2010 Jan 22;6(1):e1000736. PMID: 20107515

207. Gehlenborg N, Hwang D, Lee IY, Yoo H, Baxter D, Petritis B, Pitstick R, Marzolf B,

DeArmond SJ, Carlson GA, Hood L. The Prion Disease Database: a comprehensive Transcriptome resource for systems biology research in prion diseases. Database (Oxford). 2009; 2009:bap011. Epub 2009 Sep 17. PMID: 20157484

208. Narvid J, Gorno-Tempini ML, Slavotinek A, DeArmond SJ, Cha YH, Miller BL, Rankin K. Of

brain and bone: the unusual case of Dr. A. Neurocase. 2009 Jun; 15(3):190-205. PMID: 20183548


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209. DeArmond SJ, Bajsarowicz K. PrPSc accumulation in neuronal plasma membranes links

Notch-1 activation to dendritic degeneration in prion diseases. Mol Neurodegener. 2010 Jan 21; 5:6. PMID: 20205843.

210. Stanker LH, Serban AV, Cleveland E, Hnasko R, Lemus A, Safar J, DeArmond SJ, Prusiner

SB. Conformation-Dependent High-Affinity Monoclonal Antibodies to Prion Proteins. J Immunol. 2010 Jun 7 (Epub ahead of print). PMID: 20530267.

211. Boxer AL, Mackenzie IR, Boeve BF, Baker M, Seeley WW, Crook R, Feldman H, Hsiung

GY, Tutherford N, Laluz V, Whitwell J, Foti D., McDade E, Molano J, Karydas A, Wojtas A, Goldman J, Mirsky J, Sengdy P, DeArmond S, Miller BL, Rademakers R. Clinical, neuroimaging and neuropathologyical features of a new chromosome 9p-linked FTD-ALS family. J Neurol Neurosurg Psychiatry. 2010 Jun 20. (Epub ahead of print). PMID: 20562461

212. Zou WQ, Puoti G, Xiao X, Yuan J, Qing L, Cali I, Shimoji M, Langeveld JP, Castellani R,

Notari S, Crain B, Schmidt RE, Geschwind M, Dearmond SJ, Cairns NJ, Dickson D, Honig L, Torres JM, Mastrianni J, Capellari S, Giaccone G, Belay ED, Schonberger LB, Cohen M, Perry G, Kong Q, Parchi P, Tagliavini F, Gambetti P. Variably protease-sensitive prionopathy: a new sporadic disease of the prion protein. Ann Neurol. 2010 Aug;68(2):162-72.PMID: 20695009.

213. Giles K, Glidden DV, Patel S, Korth C, Groth D, Lemus A, DeArmond SJ, Prusiner SB.

Human prion strain selection in transgenic mice. Ann Neurol. 2010 Aug;68(2):151-61.PMID: 20695008

214. Tartaglia MC, Thai JN, See T, Kuo A, Harbaugh R, Raudabaugh B, Cali I, Sattavat M,

Sanchez H, DeArmond SJ, Geschwind MD. Pathologic evidence that the T188R mutation inPRNP is associated with prion disease. J. Neuropathol Exp Neurol. 2010 Dec;69(12):1220-7. PMID:21107135.

215. Watts JC, Giles K, Grillo SK, Lemus A, DeArmond SJ, Prusiner SB. Bioluminescence

imaging of A{beta} deposition in bigenic mouse models of Alzheimer's disease. Proc Natl Acad Sci USA. 2011 Jan 24. PMID: 21262831.

216. Rohrer JD, Geser F, Zhou J, Gennatas ED, Sidhu M, Trojanowski JQ, DeArmond SJ, Miller

BL, Seeley WW. TDP-43 subtypes are associated with distinct atrophy patterns in frontotemporal dementia. Neurology. 2010 Dec 14;75(24):2204-11. PMID: 21172843.

217. Watts JC, Giles K, Grillo SK, Lemus A, DeArmond SJ, Prusiner SB. Bioluminescence

imaging of Abeta deposition in bigenic mouse models of Alzheimer's disease. Porc Natl Acad Sci USA. 2011 Feb 8; 108(6):2528-33. Epub 2011 Jan 24. PMID: 21262831.

218. Kim EJ, Sidhu M, Gaus SE, Huang EJ, Hof PR, Miller BL, DeArmond SJ, Seeley WW.

Selective frontoinsular von economo neuron and fork cell loss in early behavioral variant frontotemporal dementia. Cereb Cortex. 2011 Jun 14. [Epub ahead of print] PMID: 21653702.

219. Safar JG, Giles K, Lessard P, Letessier F, Patel S, Serban A, DeArmond SJ, Prusiner SB.

Conserved properties of human and bovine prion strains on transmission to guinea pigs. Lab Invest. 2011 Sep;91(9):1326-36. Doi: 10.1038/labinvest.2011.89. Epub 2011 Jul 4. [PubMed - in process] PMID: 21727894.


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220. Lee SE, Rabinovici GD, Mayo MC, Wilson SM, Seeley WW, DeArmond SJ, Huang EJ,

Trojanowski JQ, Growdon ME, Jang JY, Sidhu M, See TM, Karydas AM, Gorno-Tempini ML, Boxer AL, Weiner MW, Geschwind MD, Rankin KP, Miller BL. Clinicopathological correlations in corticobasal degeneration.Ann Neurol. 2011 Aug;70(2):327-40. doi: 10.1002/ana.22424. PMID: 21823158.

221. Ghaemmaghami S, Watts JC, Nguyen HO, Hayashi S, DeArmond SJ, Prusiner SB.

Conformational transformation and selection of synthetic prion strains. J Mol Biol. 2011 Aug 4 [Epub ahead of print] PMID: 21839745.

222. Rankin KP, Mayo MC, Seeley WW, Lee S, Rabinovici G, Gorno-Tempini ML, Boxer AL,

Weiner MW, Trojanowski JQ, DeArmond SJ, Miller BL. Behavioral variant frontotemporal dementia with corticobasal degeneration pathology: phenotypic comparison to bvFTD with Pick's Disease. J Mol Neurosci. 2011 Sep 1. [Epub ahead of print] PMID:21881831.

Review Articles

1. DeArmond SJ The molecular pathology of prion diseases. West J Med (Epitome)149-594,

11/1988.

2. Prusiner SB, DeArmond SJ Prion diseases and neurodegeneration. Annu Rev Neurosci

17: 311-39, 1994.

3. DeArmond SJ, Yang SL, Cayetano-Canlas J, Groth D, Prusiner SB The neuropathological

phenotype in transgenic mice expressing different prion protein constructs. Philos Trans R Soc Lond B Biol Sci 343: 415-23, Mar/29/1994.

Books and Chapters

1. Books

2. DeArmond SJ, Fusco MM, Dewey M. Structure of the Human Brain, a Photographic Atlas,

First Ed. Oxford Press, New York.

3. DeArmond SJ, Fusco MM, Dewey MM. Structure of the Human Brain, a Photographic

Atlas (with an Atlas of Computerized Brain Tomography), Second Ed. Oxford University Press, New York.

4. DeArmond SJ, Fusco MM, Dewey MM. Structure of the Human Brain, Third Ed. Oxford

University Press, New York.

5. Künzle-Watson K, DeArmond SJ. Ice Skating: Steps to Success. Human Kinetics Press,

Champaign, IL.

6. Chapters

7. Eng LF, DeArmond SJ. Glial fibrillary acidic (GFA) protein immunocytochemistry in

development and neuropathology. Editor(s): Fedoroff S, Hertz L (Eds),. Proceedings of the XI International Congress of Anatomy, Part A: Alan R. Liss, New York. 65-79, 1981.


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8. Eng LF, DeArmond SJ. Immunocytochemical studies of astrocytes in normal development

and disease. Editor(s): Federoff S, Hertz L. Advances in Cellular Neurobiology: Academic Press, New York. 3: 145-171, 1982.

9. Eng LF, DeArmond SJ. Immunochemistry of the glial fibrillary acidic (GFA) protein.

Editor(s): Zimmerman HM. Progress in Neuropathology: Raven Press, New York. 5: 19-39, 1983.

10. DeArmond SJ, Eng LF. Immunohistochemistry: Techniques and application to

neurooncology. Editor(s): Rosenblum ML, Wilson CB. Progress in Experimental Brain Tumor Research: Brain Tumor Biology: Karger Medical and Scientific Publishers, Basel, Switzerland. 1: 92-117, 1984.

11. McKinley MP, Barry RA, Braunfeld MB, Prusiner SB, DeArmond SJ. Ultrastructural and

immunological investigations of scrapie prions. Editor(s): Scheibel AB, Wechsler AF, Brazier MAB. The Biological Substrates of Alzheimer's Disease, UCLA Forum Medical Sciences: Academic Press, New York. 27: 145-160, 1986.

12. DeArmond SJ, Kretzschmar HA, McKinley MP, Prusiner SB. The molecular pathology of

prion diseases. Editor(s): Prusiner SB, McKinley MP. Prions: Novel Infectious Pathogens Causing Scrapie and Creutzfeldt-Jakob Disease: Academic Press, New York. 387-414, 1987.

13. DeArmond SJ, Nagashima T, Cho KG, Murovic JA, Davis RL, Hoshino TL. Correlation of in

situ cell kinetics and degree of anaplasia in human brain tumors: Identification of S-phase cells with anti-BUdR antibodies. Editor(s): Chatel M, Darcel F, Pecker J. Brain Oncology: Martinus Nijhoff Publishers, Dordrecht, The Netherlands. 67-74, 1987.

14. Prusiner SB, Stahl N, DeArmond SJ. Novel mechanisms of CNS degeneration - prion

structure and biology. Novel Infectious Agents and the Central Nervous System, Ciba Foundation Symposium No 135: John Wiley and Sons, London. 239-256, 1988.

15. Prusiner SB, Hsiao KK, Bredesen DE, DeArmond SJ. Prion diseases. Editor(s): Vinken PJ,

Bruyn GW, Klawans HL, McKendall RR. Handbook of Clinical Neurology: Elsevier, Amsterdam, Netherlands. 12: 543-580, 1989.

16. DeArmond SJ, Gonzales MF. Immunological and molecular studies of dementia.

Proceedings of the Alzheimer's Disease Workshop, 3rd International Conference, 1988. Australian J of Ageing: Allanby Press Pty. Ltd., Melbourne, Australia. 100-104, 1989.

17. Prusiner SB, DeArmond SJ. Prion diseases of the central nervous system. Editor(s):

Cancilla PA, Vogel SF, Kaufman. International Academy of Pathology, Monographs in Pathology: Williams and Wilkins, Baltimore. 86-122, 1989.

18. Gabizon R, McKinley MP, Groth D, Westaway D, DeArmond SJ, Carlson GA, Prusiner SB.

Immunoaffinity purification and neutralization of scrapie prions. Editor(s): Iqbal K, Wisniewski HM, Winblad B. Alzheimer's Disease and Related Disorders: Alan R. Liss, Inc., New York. 583-600., 1989.

19. DeArmond SJ, Gonzales M, Mobley WC, Kon AA, Stern H, Prusiner SB. PrPSc in scrapie

infected hamster brain is spatially and temporally related to histopathology and infectivity titer. Editor(s): Iqbal K, Wisniewski HM, Winblad B. Alzheimer's Disease and Related Disorders: Alan R. Liss, Inc., New York. 601-618, 1989.


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20. Prusiner SB, Carlson GA, DeArmond SJ, Gabizon R, Hsiao K, Scott M, Stahl N, Westaway

D. Transmissable and genetic prion diseases of humans and animals. Editor(s): Rosenberg R, Prusiner SB, DiMauro S, Bachi R, Kunkel L. The Molecular and Genetic Basis of Neurological Disease : Butterworth Publishers, Stoneham, MA. 585-601, 1992.

21. DeArmond SJ, Jendroska K, Yang S-L, Taraboulos A, Hecker R, Hsiao K, Stowring L, Scott

M, Prusiner SB. Scrapie prion protein accumulation correlates with neuropathology and incubation times in hamsters and transgenic mice. Editor(s): Prusiner S, Collinge J, Powell J, Anderton B. Prion Diseases of Humans and Animals: Ellis Horwood, New York. 483-496, 1993.

22. DeArmond SJ, Prusiner SB. Prion diseases. Editor(s): Bloom FE, Kupfer DJ.

Psychopharmacology: The Fourth Generation of Progress: Raven Press, New York. 1521-1530, 1995.

23. DeArmond SJ, Prusiner SB. Prions, 10 Ed. Editor(s): Damjanov I, Linder J. Anderson's

Pathology: Mosby, St. Louis. 1042-1060., 1996.

24. DeArmond SJ, Prusiner SB. Neurodegeneration: Prion diseases, 6th Ed. Editor(s): Graham

D, Lantos P. Greenfield's Neuropathology: Arnold, London. 235-280, 1996.

25. DeArmond SJ, Qiu Y, Wong K, Nixon R, Hyun W, Prusiner SB, Mobley WC. Abnormal

plasma membrane properties and functions in prion-infected cell lines. Cold Spring Harbor Symposia on Quantitative Biology : Cold Spring Harbor Laboratory Press, Cold Spring Harbor. LXI: 531-540, 1996.

26. DeArmond SJ, Prusiner SB. Molecular neuropathology of prion disease, 2nd Ed. Editor(s):

Rosenburg RN, Prusiner SB, DiMauro S, Barchi RL. The Molecular and Genetic Basis of Neurologic Disease: Butterworth Heinemann, Stoneham, MA. 145-163, 1997.

27. DeArmond SJ, Dickson DW, DeArmond B. Degenerative diseases of the central nervous

system. Editor(s): Davis RL, Robertson DM. Textbook of Neuropathology: Williams and Wilkins, Baltimore. 1063-1178, 1997.

28. Clark AW, DeArmond SJ, Mann DMA, Tateishi J. Creutzfeldt-Jakob disease and the prion

hypothesis. Editor(s): Clark AW, Dickson DW. The Primary Degenerative Dementias Other than Alzheimer's Disease: Carolina Academic Press, 1998.

29. DeArmond SJ, Prusiner SB. Prion Diseases. Editor(s): Markesbery WR. Neuropathology of

Dementing Disorders: Arnold Press, London. 340-376, 1998.

30. DeArmond SJ. Prion diseases: the spectrum of etiologic and pathogenic mechanisms.

Editor(s): Folstein, MF. Neurobiology of Primary Dementia: American Psychiatric Press, Washington, DC. 83-118, 1998.

31. Prusiner SB, Safar J, Cohen FE, DeArmond SJ. The Prion Diseases, 2nd Ed. Editor(s):

Terry, RD, Katzman, R, Bick, LK, Sisodia, SS. Alzheimer Disease: Lippincott Williams & Wilkins, Philadelphia. 161-179, 1999.

32. DeArmond SJ, Ironside JW. Neuropathology of Prion Diseases. Prion Biology and

Diseases: Cold Spring Harbor Laboratory Press, Cold Spring Harbor. 585-652, 1999.


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33. DeArmond SJ. Differential targeting of neurons by prion strains. Editor(s): Baker HF.

Methods in Molecular Medicine: Molecular Pathology of the Prions: Human Press Inc., Totowa, NJ. 59: 85-110, 2001.

34. DeArmond SJ, Kretzschmar HA, Prusiner SB. Prion Diseases, Seventh Ed. Editor(s):

Graham, DI and Lantos, PL. Greenfield's Neuropathology: Arnold, London. 273-323, 2002.

35. DeArmond SJ and Prusiner SB. Perspectives on prion biology, prion disease pathogenesis,

and pharmacological approaches to treatment. Editor(s): Ghetti, B and Piccardo, P. Prion Diseases, Clinics in Laboratory Medicine: WB Saunders Company, Philadelphia. 23: 1-41, 2003.

36. DeArmond SJ, Simko JP, Gaskin DA. The molecular and genetic basis of

neurodegenerative diseases. Editor(s): Weidner, N, Cote, RJ, Suster, S, Weiss, LM. Modern Surgical Pathology: WB Saunders Co., Philadelphia, PA. 2121-2153, 2003.

37. DeArmond SJ and Prusiner SB. Prion Diseases. Editor(s): Rosenberg RN, Prusiner SB,

DiMauro S, Barchi RL, Nestler EJ. The Molecular and Genetic Basis of Neurologic and Psychiatric Disease: Butterworth-Heinemann, U.S.A. 151-178, 2003.

38. DeArmond SJ, Ironside JW, Bouzamondo-Bernstein E, Peretz D, Fraser JR.

Neuropathology of Prion Diseases, 2nd Ed. Editor(s): Prusiner, SB. Prion Biology and Diseases: Cold Spring Harbor Laboratory Press, Cold Spring Harbor, NY. 143-186, 2004.

39. Prusiner SB, Safar J, DeArmond SJ. Bioassays of Prions, 2nd Ed. Editor(s): Prusiner, SB,

Ed. Prion Biology and Diseases: Cold Spring Harbor Laboratory Press, Cold Spring Harbor, NY. 143-186, 2004.

40. Prusiner SB, Scott MR, DeArmond SJ, Carlson G. Transmission and replication of prions,

2nd Ed. Editor(s): Prusiner, SB. Prion Biology and Diseases: Cold Spring Harbor Laboratory Press, Cold Spring Harbor, NY. 187-242, 2004.

41. Scott M, Peretz D, Ridley RM, Baker HF, DeArmond SJ, Prusiner SB. Transgenetic

investigations of the species barrier and prion strains, 2nd Ed. Editor(s): Prusiner, SB. Prion Biology and Diseases: Cold Spring Harbor Laboratory Press, Cold Spring Harbor, NY. 435-482, 2004.

42. Harris DA, Peters PJ, Taraboulos A, Lingappa V, DeArmond SJ, Prusiner SB. Cell biology

of prions, 2nd Ed. Editor(s): Prusiner, SB. Prion Biology and Diseases: Cold Spring Harbor Laboratory Press, Cold Spring Harbor, NY. 483-544, 2004.

43. Harris DA, Peters PJ, Taraboulos A, Lingappa V, DeArmond SJ, Prusiner SB. Some

strategies and methods for the study of prions, 2nd Ed. Editor(s): Prusiner, SB. Prion Biology and Diseases: Cold Spring Harbor Laboratory Press, Cold Spring Harbor, NY. 857-920, 2004.

44. Harris DA, Peters PJ, Taraboulos A, Lingappa V, DeArmond SJ, Prusiner SB. Biosafety

issues in prion diseases, 2nd Ed. Editor(s): Prusiner, SB. Prion Biology and Diseases: Cold Spring Harbor Laboratory Press, Cold Spring Harbor, NY. 921-960, 2004.

45. Legname G, DeArmond SJ, Cohen FE, Prusiner SB. Pathogenesis of Prion Diseases.

ProteinReviews, Vol. 6: Protein misfolding, aggregataion, and conformational diseases. Part B:


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Molecular mechanisms of conformational diseases. Editor(s) Uversky VN and Fink A. Springer Science+Business Media, LLC, NY. 125-146, 2007.

46. Tousseyn TA, Simko JP, Gaskin DA, DeArmond SJ. The Molecular and Genetic Basis of

Neurodegenerative Diseases. Editor(s): Weidner, N, Cote, RJ, Suster, S, Weiss, LM. Modern Surgical Pathology: WB Saunders Co., Philadelphia, PA. Volume 2: 2039-2068. 2009.

Other Publications

1. DeArmond SJ Annual Meeting of the American Association of Neuropathologists Chicago,

IL 06/2001 Syllabus: Special Course, Developmental Biology 2001

2. De Armond SJ, Fusco MM. The effect of preoptic warming on the arousal system of the

mesencephalic reticular formation. Exp Neurol. 33: 653-70, Dec/1971.

3. de Armond SJ, Eng LF, Rubinstein LJ. The application of glial fibrillary acidic (GFA) protein

immunohistochemistry in neurooncology. A progress report. Pathol Res Pract. 168: 374-94, 1980.

4. De Armond SJ, Eng LF. Immunohistochemistry: techniques and application to

neurooncology. Prog Exp Tumor Res. 27: 92-117, 1984.

5. Liu HC, De Armond SJ, Edwards MS. An unusual spinal meningioma in a child: case

report. Neurosurgery. 17: 313-6, Aug/1985.

6. Koch TK, Berg BO, De Armond SJ, Gravina RF. Creutzfeldt-Jakob disease in a young

adult with idiopathic hypopituitarism. Possible relation to the administration of cadaveric human growth hormone. N Engl J Med. 313: 731-3, Sep/19/1985.

7. Rutka JT, Kleppe-Hoifodt H, Emma DA, Giblin JR, Dougherty DV, McCulloch JR, De

Armond SJ, Rosenblum ML. Characterization of normal human brain cultures. Evidence for the outgrowth of leptomeningeal cells. Lab Invest. 55: 71-85, Jul/1986.

8. Sze G, De Armond SJ, Brant-Zawadzki M, Davis RL, Norman D, Newton TH. Foci of MRI

signal (pseudo lesions) anterior to the frontal horns: histologic correlations of a normal finding. AJR Am J Roentgenol. 147: 331-7, Aug/1986.

9. Murovic JA, De Armond S, Nagashima T, Edwards MS, Hoshino T. Cell kinetics analysis in

a case of teratoma of the thoracic spine. J Neurosurg. 65: 331-4, Sep/1986.

10. Germano IM, Pitts LH, Berry I, De Armond SJ. High energy phosphate metabolism in

experimental permanent focal cerebral ischemia: an in vivo 31P magnetic resonance spectroscopy study. J Cereb Blood Flow Metab. 8: 24-31, Feb/1988.

11. Cho KG, De Armond SJ, Barnwell S, Edwards MS, Hoshino T. Proliferative characteristics

of intracranial and spinal tumors of developmental origin. Cancer. 62: 740-8, Aug/15/1988.

12. Rutka JT, De Armond SJ, Giblin J, McCulloch JR, Wilson CB, Rosenblum ML. Effect of

retinoids on the proliferation, morphology and expression of glial fibrillary acidic protein of an anaplastic astrocytoma cell line. Int J Cancer. 42: 419-27, Sep/15/1988.


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13. De Armond SJ, Gonzales M, Mobley WC, Kon AA, Stern A, Prusiner H, Prusiner SB.

PrPSc in scrapie-infected hamster brain is spatially and temporally related to histopathology and infectivity titer. Prog Clin Biol Res. 317: 601-18, 1989.

14. Schendel SA, Pearl RM, De Armond SJ. Pathophysiology of cleft lip muscles following the

initial surgical repair. Plast Reconstr Surg. 88: 197-200, Aug/1991.

15. Martindale J, Geschwind MD, De Armond S, Young G, Dillon WP, Henry R, Uyehara-Lock

JH, Gaskin DA, Miller BL. Sporadic Creutzfeldt-Jakob disease mimicking variant Creutzfeldt-Jakob disease. Arch Neurol. 60: 767-70, May/2003.

ABSTRACTS

1. DeArmond SJ, Fusco MM "Electrophysiological and behavioral evidence for linkage between

thermoregulatory and sleep-wake mechanisms." Fed Proc. (28) : 587, 1969.

2. DeArmond SJ, Fusco MM "The effect of preoptic warming on the mesencephalic reticular

formation." Fed Proc. (30) : 213, 1971.

3. Hess JR, Vandenberg SR, Herman MM, DeArmond SJ, Miller MH, Rubinstein LJ "Melanin

as a marker for the selection of enriched primitive neuroepithelial cell populations in a mouse teratoma." J Neuropathol Exp Neurol. (38) : 320, 1979.

4. Eng LF, DeArmond SJ, Deibler GE, Bacon M, Kies MW "A neurochemical and

immunocytochemical study of P2 protein." Trans Am Soc Neurochem 11:156, 1980. (11) : 156, 1980.

5. DeArmond SJ, Deibler GE, Bacon M, Kies MW, Eng LF "An immunocytochemical study of

P2 protein in the human." J Neuropathol Exp Neurol. (39) : 338, 1980.

6. Sobel RA, DeArmond SJ, Forno LS, Eng LF "Glial fibrillary acidic protein in hepatic

encephalopathy." J Neuropathol Exp Neurol. Vol. 40: 322, 1981.

7. DeArmond SJ "Turnover of glial fibrillary acidic protein in vivo." Veterans Administration

Regeneration Research Symposium, Portland, OR. 08/1981.

8. DeArmond SJ, Lee YL, Eng LF "Turnover of GFA protein in jimpy and normal mouse spinal

cords." J Neuropathol Exp Neurol. Vol. 41: 359, 1982.

9. DeArmond SJ, Lee YL, Eng LF "Turnover of glial fibrillary acidic protein in the

mouse.(Symposium on Astrocytes)." J Neurochem. Vol. 41(suppl): S3, 1983.

10. Kretzschmar HA, DeArmond SJ "An ELIZA and transblot study of GFAP in hepatic

encephalopathy." J Neuropathol Exp Neurol. Vol. 43: 323, 1984.


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11. Michie S, DeArmond SJ "The behavior of GFAP fractions with in situ postmortem autolysis

of mouse spinal cord." J Neuropathol Exp Neurol. Vol. 43: 311, 1984.

12. DeArmond SJ, Kretzschmar HA, McKinley MP, Barry MB, Braunfeld MB, Prusiner SB

"Identification of prion filaments in scrapie infected brain." J Neuropathol Exp Neurol. Vol. 44: 364, 1985.

13. Hoshino T, Nagashima T, Murovic JA, DeArmond SJ, Davis RL "Immunohistochemical

demonstration of S phase cells in human brain tumor tissues exposed to bromodeoxyuridine (BrdU) in situ." J Neuropathol Exp Neurol. Vol. 44: 316, 1985.

14. Nagashima T, Hoshino T, Murovic J, DeArmond SJ, Wilson SB "A cell kinetics study of

human brain tumors in situ using bromodeoxyuridine (BUdR)." Amer Assoc Neurol Surgeons (53rd Annual Meeting, Atlanta, GA). 04/1985.

15. McKinley MP, Barry RA, DeArmond SJ, Braunfeld MB, Prusiner SB "Amyloid plaques in

scrapie-infected hamster brain contains filaments composed of the prion protein PrP 27-30." Amer Soc Virology (Annual Meeting), Albuquerque, NM. 07/1985.

16. Hoshino T, Nagashima T, Cho KG, Murovic JA, Zhang R, Davis RL, DeArmond SJ

"Proliferative potential of malignant and non-malignant meningiomas." J Neuropathol Exp Neurol. Vol. 45: 320, 1986.

17. DeArmond SJ, Kretzschmar H, McKinley M, Barry R, Meyer R, Westaway D, Prusiner S

"Chemical and biological properties of the prion protein and its cellular isoform." J Neuropathol Exp Neurol. Vol. 45: 329, 1986.

18. DeArmond SJ, Kretzschmar HA, Stowring LE "Gliofibrillogenesis in prion disease." Am Soc

for Neurochemistry, 17th Annual Meeting, Montreal, Canada. 03/1986.

19. Kretzschmar H, Stowring L, Westaway DA, Prusiner SB, DeArmond SJ "Cloning and

sequencing of a human prion protein cDNA." Am Soc for Virology (Annual Meeting), UC, Santa Barbara. 06/1986.

20. DeArmond SJ, Davis RL, Hoshino T "Correlation of in situ cell kinetics and degree of

anaplasia in human brain tumors: Identification of S-phase cells with anti-BUdR antibodies." Int Meeting on Brain Oncology, Rennes, France. 09/1986.

21. Kretzschmar HA, Stowring LE, Westaway D, Stubblbine SB, Prusiner SB, DeArmond SJ

"Molecular cloning and sequencing of a human prion protein cDNA. X Int Congr Neuropathol, Stockholm, Sweden." Abstract #336. 09/1986.

22. Hoshino T, Nagashima T, Cho KG, Murovic JA, Davis RL, DeArmond SJ "Proliferative

potential of human brain tumors in situ measured by monoclonal antibody against bromodeoxyuridine." X Int Congr Neuropathol, Stockholm, Sweden, Abstract #908. 09/1986.

23. Barry RA, DeArmond SJ, Prusiner SB "Immunological studies of scrapie prions and

generation of monoclonal antibodies to PrP." II Symposium Internationale sur les Virus Nonconventionnels du Systeme Nerveux Central, Paris, France. 12/1986.


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24. Koo EH, DeArmond SJ, Hart J, Gordon B, Selkoe DJ, Price DL "Cerebellar plaques in

presenile dementia: Gerstmann-Straussler syndrome or Alzheimer's disease?." J Neuropathol Exp Neurol. Vol. 46: 338 (#19), 1987.

25. Hoshino T, Nagashima T, Cho KG, Davis RL, DeArmond SJ "Proliferative potential of

human neuroepithelial tumors in situ." J Neuropathol Exp Neurol. Vol. 46: 372 (#121), 1987.

26. DeArmond SJ, Mobley WC, Renaut P, Prusiner SB "Changes in the localization of brain

prion proteins during scrapie infection." J Cell Biol (Abstracts). Vol. 105: 316a, 1987.

27. Mobley WC, DeArmond SJ, Prusiner SB, Johnston MV, McKinley MP "Nerve growth factor

stimulates prion protein gene expression in developing brain." Society for Neuroscience, Annual Meeting, New Orleans, LA. . 12/1987.

28. Charman H, Gonzales M, Renaut P, DeArmond SJ "Astrocytes in scrapie, Creutzfeldt-Jakob

disease, and non-neoplastic conditions contain a ras P21 protein." J Neuropathol Exp Neurol. Vol. 47: 345, 1988.

29. DeArmond SJ, Mobley WC, Gonzales M, Renaut P, Charman H, Prusiner SB "Brain PrPSc

in hamster is spatially and temporally related to scrapie histopathology and infectivity titer." J Neuropathol Exp Neurol. Vol. 47: 345, 1988.

30. Gonzales MF, Renaut P, Stowring L, Charman HP, Prusiner SB, DeArmond SJ "Scrapie

prion protein promotes GFAP and vimentin synthesis in vitro." J Neuropathol Exp Neurol. Vol. 47: 345, 1988.

31. Coufal E, Jendroska K, Oesch B, Prusiner SB, DeArmond SJ "Autoradiographic evidence of

PrPSc binding sites in the hippocampus." J Neuropathol Exp Neurol. Vol. 49: 290, 1990.

32. Serban D, Taraboulos A, DeArmond SJ, Prusiner SB "Rapid detection of Creutzfeldt-Jakob

disease and scrapie prion proteins." J Neuropathol Exp Neurol. Vol. 49: 290, 1990.

33. Jendroska K, Stowring L, Prusiner SB, DeArmond SJ "Comparative neuropathology,

neurochemistry, and molecular biology of scrapie in Syrian, Armenian, and Chinese hamsters.: Comparative neuropathology, neurochemistry, and molecular biology of scrapie in Syrian, Armenian, and Chinese hamsters." J Neuropathol Exp Neurol. Vol. 49: 290, 1990.

34. Scott M, Foster D, Mirenda C, Serban D, Coufal F, Wälchi M, Torchia M, Groth D, Carlson

G, DeArmond SJ, Westaway D, Prusiner SB "Transgenic mice expressing hamster prion protein produce series-specific scrapie infectivity and amyloid plaques." J Neuropathol Exp Neurol. Vol. 49: 311, 1990.

35. McKinley MP, Taraboulos A, Kenaga L, Serban D, DeArmond SJ, Stieber A, Prusiner SB,

Gonatas N "Ultrastructural localization of scrapie prion proteins in secondary lysosomes of infected cultured cells (Abst #1760)." American Society for Cell Biology, 30th Annual Meeting, San Diego, CA. 12/1990.

36. DeArmond SJ, Kenaga L, Yang SL, Serban D, Caytano SJ, Prusiner SB, McKinley MP

"Immunohistochemical studies of prion proteins deposited in scrapie amyloid plaques using monospecific antibodies against PrP synthetic peptides (Abst #2776)." American Society for Cell Biology, 30th Annual Meeting, San Diego, CA. 12/1990.


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37. DeArmond SJ, Hecker R, Tarabolous A, Scott M, Yang SL, Pan K-M, Torchia M, Prusiner

SB "Scrapie prion replication is brain-region-specific in transgenic mice and hamsters." Cold Spring Harbor Symposium, Molecular Biology of Aging, Cold Spring Harbor, New York. 04/1992.

38. Qui Y, Ninchak-Casey A, Yehiely F, Prusiner SB, DeArmond SJ "Brain region differences in

prion protein structure and selective vulnerability of neurons in scrapie." J Neuropathol Exp Neurol. Vol. 54: 414, 1995.

39. Wong K, Qui Y, VanCleff J, Prusiner SB, DeArmond SJ "Scrapie prions caused decreased

membrane fluidity in cultured cells." J Neuropathol Exp Neurol. Vol. 54: 416, 1995.

40. Enns GM, Hopper CL, DeArmond SJ, Bass NE, Zanko A, Weisiger K, Ohnstad C, Bolabi M,

Packman S "Type I fiber predominance can be caused by isolated mitochondrial respiratory chain complex I deficiency." Am. J. Hum. Genet. Vol. 63: A266, 1998.

41. Bouzamondo E, Spilman P, Ralston HJ, Prusiner SB, DeArmond SJ "Abnormalities of

plasma membrane structure and function in scrapie-infected Syrian hamsters." 28th Annual Meeting of Neuroscience, Los Angeles, CA. 11/1998.

42. Supattapone S, Muramoto Tagaard C, Peretz D, Willie H, Ngyuen O, Cohen FE, DeArmond

SJ, Prusiner SB, Scott M "Propagation of infectious miniprions in transgenic mice expressing truncated and partially deleted PrP molecules." Am Soc of Cell Biology Meeting, San Francisco, CA. 12/1998.

43. Bouzamondo E, Milroy AM, Lisse T, Ralston HJ, Prusiner SB, DeArmond SJ "Impairment of

the GABAergic system during scrapie infection in hamsters: Insights from functional and structural studies." 29th Annual Meeting of the Society of Neuroscience, Miami, FL. 11/1999.

44. Ishikura N, Huang EJ, Clever JL, Samayoa E, Tang AA, DeArmond SJ "Programmed

dendritic atrophy in Prion disease." J. Neuropathol. Expt. Neurol. Vol. 63 (No. 18): 514, 2004.

45. Bouzamondo-Bernstein E, Hopkins SD, Ralston HJ III, Prusiner SB, DeArmond SJ "New

functional, structural and ultrastructural insights on the GABAergic system during scrapie: Relevance for neurodegenerative mechanisms." 6th International Symposium on NeuroVirology (No I.18). 09/2004.

RESEARCH PROGRAM

I began the Neuropathology Research Laboratory at UCSF in 1983 with studies of astrocyte

biology and gliomas. In 1984, Stanley Prusiner asked me to add a neuropathology component to

his studies of prion disease, which led to a very productive 27 years of collaboration, which still

continues. My laboratory's main contributions to the study of prion diseases are: (1) The first to

show that amyloid plaques in scrapie and Creutzfeldt-Jakob disease are composed of abnormal

prion protein (PrPSc). (2) The first to show that there is significantly more non-amyloid PrPSc

accumulation in the brain than amyloid and that the former is linked to neuronal degeneration in

prion diseases. That study was published in Neurology in 1987. In 1998, two past editors of

Neurology chose that 1987 publication as the first of a set of "landmark" articles for the 50th


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anniversary of the American Academy of Neurology (Landmark Articles. Neurology 50:1-2ff,

1998). (3) My lab was the first to show that PrPSc accumulates in the plasma membrane of

scrapie-infected cell lines and, in doing so, has a profound effect on receptor-mediated

transmembrane signaling of the type that can cause neuronal dysfunction and degeneration. (4)

The first to show that each prion strain targets different neuron populations in the brain for

conversion of PrPC to nascent PrPSc. (5) The first to show that the neuron-specific variations in

the structure of the two Asn-linked oligosaccharides attached to the normal prion protein, PrPC,

modulate the interaction of an infecting PrPSc with PrPC and, in doing so, is the basis of

differential neuronal targeting by prion strains (DeArmond et al., PrPC glycoform heterogeneity

as a function of brain region: Implications for selective targeting of neurons by prion strains. J

Neuropathol. Expt. Pathol. 58:1000-1009, 1999). (6) During 24 years of neurohistological

analysis of animal prion diseases, my Lab has generated multiple lines of data that support the

hypothesis that prion strain-determined variations in the disease phenotype are determined

almost exclusively by structural and conformational differences between the prion's PrPSc and

the host's PrPC (summarized in: DeArmond, Kretzschmar, and Prusiner. Prion Diseases. In:

Greenfield's Neuropathology, Seventh Edition, (Graham, DI and Lantos, PL, eds) Arnold,

London, pp 273-323, 2002); DeArmond SJ, Bajsarowicz K: PrPSc accumulation in neuronal

plasma membranes links Notch-1 activation to dendritic degeneration in prion diseases. Mol

Neurodegener. 2010). (7) Our lab has discovered many new prion disorders in transgenic mice

expressing different PrP constructs (for example see: Supattapone et al. A protease-resistant 61

residue prion peptide causes neurodegeneration in transgenic mice. Mol. & Cell Biol. 21:2608-

2616, 2001). Because of our contributions, Stanley Prusiner gave special recognition to my

Neuropathology Research Laboratory for its contribution to his 1997 Nobel Prize in Medicine.

We have acquired direct evidence to support our hypothesis of a cause-effect

relationship between PrPSc accumulation in neurons and abnormalities of neuron-specific

physiological functions and neuropathological changes by studying animal models of prion

disease acquired by infection and in genetically determined prion diseases. (1) In a complex

quantitative functional, neurochemical, ultrastructural, and neuropathological changes during the

course of laboratory scrapie in hamsters, we discovered a stereotypical progression of functional

and morphological changes in prion diseases that is transmitted anterogradely from one brain

region to another along neuroanatomical pathways (Bouzamondo-Bernstein et al.: The

neurodegeneration sequence in prion diseases: Evidence from functional, morphological and

ultrastructural studies J. Neuropathol. Expt. Neurol. 63:882-899, 2004). In brief, the

neurodegenerative sequence in a brain region begins with anterograde trafficking of PrPSc to its

nerve terminals. This is followed in one to two weeks by decreased release of neurotransmitters


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from axon terminals, by degeneration of presynaptic nerve terminals, by transynaptic spread of

PrPSc formation to the postsynaptic neuron, and by dendritic atrophy. Nerve cell body

degeneration and death follow these events by one to two months. (2) The results of that study

have focused our research now on synaptic dysfunction and degeneration because they are

early events, account for many clinical features in human prion diseases, and are potentially

reversible. Specifically, we have been testing whether PrPSc accumulation upregulates some

genes important to synapse integrity. That is, is synaptic degeneration a programmed event (i.e.

synaptic apoptosis) analogous to programmed nerve cell death (apoptosis). We began our

studies by examining genes that regulate growth of presynaptic boutons and dendrites during

embryonic development of the CNS. We found that accumulation of PrPSc is associated with

increased Notch-1 expression, a gene product known to cause regression of axons and

dendrites during embryologic development. More importantly, PrPSc accumulation was found to

be highly correlated with cleavage of Notch-1 releasing its intracellular domain (NICD). NICD is

a transcription factor, which activates the Hes family of genes that block the expression of pro-

neuronal genes. This is the first study to link accumulation of PrPSc to molecular events that

produce clinically relevant changes in prion diseases (Ishikura et al. Notch-1 activation and

dendritic atrophy in prion diseases PNAS 102:886-891, 2005). The DeArmond Neuropathology

Research Laboratory has just completed a drug trial in scrapie-prion infected mice (November

2006). CD-1 mice were inoculated in the thalamus with RML scrapie prions. Quinacrin (Q) alone,

-secretase inhibitor (G) alone, and quinacrine dual G+Q oral therapy was begun midway during

the incubation period. We found that G and Q work synergistically: Q helps G prevent dendritic

degeneration and G helps Q "clear" PrPSc from the brain. Dual G+Q therapy prevented axonal

transport of disease from one brain region to another (Spilman et -secretase inhibitor and

quinacrine reduce prions and prevent dendritic degeneration in murine brains. PNAS

105:10595-600, 2008). But the G component of G+Q is very toxic and can neither be used in

human nor animal studies.

It has become clear that a treatment strategy must directly target the formation of PrPC and

therefore the conversion of PrPC to PrPSc. We are currently collaborating with the Krystoff

Bankiewicz laboratory. Dr. Bankiewicz has pioneered the use of AAV vectors. With the gene

therapy, we want to decrease PrPC synthesis wherever it is occurring in the brain. Recently Dr.

Bankiewicz found that intracerebral inoculation of AAV6 targets all neurons populations in the

CNS and intraveneous inoculation of AAV9 targets all astrocytes. With these tools in hand, my

laboratory is planning to use AAV6 and AAV9 linked to PrP-shRNAs to knock down PrP

synthesis in both neurons and astrocytes throughout the CNS.


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SIGNIFICANT PUBLICATIONS

DeArmond SJ, Mobley WC, DeMott DL, Barry RA, Beckstead JH, Prusiner SB. Changes in the localization of brain prion proteins during scrapie infection. Neurology, Issue: 8. 37: 1271-80, Aug/1987. Bouzamondo-Bernstein E, Hopkins SD, Spilman P, Uyehara-Lock J, Deering C, Safar J, Prusiner SB, Ralston HJ, DeArmond SJ. The neurodegeneration sequence in prion diseases: evidence from functional, morphological and ultrastructural studies of the GABAergic system. J Neuropathol Exp Neurol, Issue: 8. 63: 882-99, Aug/2004. Ishikura N, Clever JL, Bouzamondo-Bernstein E, Samayoa E, Prusiner SB, Huang EJ, DeArmond SJ. Notch-1 activation and dendritic atrophy in prion disease. Proc Natl Acad Sci U S A, Issue: 3. 102: 886-91, Jan/18/2005. Spilman P, Lessard P, Sattavat M, Bush C, Tousseyn T, Huang EJ, Giles K, Golde T, Das P, Fauq A, Prusiner SB, DeArmond SJ. A gamma-secretase inhibitor and quinacrine reduce prions and prevent dendritic degeneration in murine brains. Proc Natl Acad Sci U S A: 10595-10600, 2008. DeArmond SJ, Bajsarowicz K. PrPSc accumulation in neuronal plasma membranes links Notch-1 activation to dendritic degeneration in prion diseases. Mol Neurodegener. 2010 Jan 21; 5:6.

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