AJ10 Ubiquitin merge

S T U

AJ10 NeuN merge

A B C

D E F

SO

D1

G8

5R

(P

-34

0)

SO

D1

G9

3A

fa

st

(P-1

36

)

J

H IG

K L

M N O

SO

D1

G9

3A

slo

w (

P-2

47

)

P Q R

Supplementary Figure 2. AJ10 immunoreactivity is associated with degenerating MNs in ventral horn both in SOD1G85R,or SOD1G93Amice (fast and slow strains). Spinal cord grey matter is delimited by a dotted line. Lumbar spinal cord sections from end-stage (postnatal (P) days in parentheses) mice overexpressing either human SOD1G85R (A-F) or SOD1G93A (G-L and M-R for the fast and slow strains , respectively) were double immunostained with AJ10 (red) and the neuronal marker NeuN (green); the large amount of AJ10-immunopositive profiles seen in (A, D, G, J and M, P), do not co-localize with apparently healthy NeuN-posi-tive neuronal cell bodies (B, C, E, F, H, I, K, L, N, O, Q, R). By contrast, AJ10 immunopositive neuronal cell bodies (encircled in J-L and P-R) no not display NeuN immunoreactivity. In (S-U) AJ10 immunoreactivity (red) was colocalized with ubiquitin (green). Some of the AJ10-positive mutant SOD1 aggregates (encircled in S-U) exhibit intense ubiquitin immunoreactivity; however, AJ10-positivity associated with neuronal processes (arrows in S-U), do not show ubiquitin immunoreactivity. Scale bar in C and F = 100 µm; U = 10 µm.