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Ks
Ks
Ks
Ks
Ks
Ks
Ks
+
+
Ks
Kr Ks
Kr
Ks
K1
Ks
+
2
+
“
+
8
Ks
β
+ β
2
Ks
Ks
β
α
α
β
“
Ks
β
Ks
+ +
+
Ks
Ks
β
≥
α β
+
Ks
Kr
Ks
Kr
Kr Ks
Kr
Ks
Ks
Ks
Kr Ks
Ks Kr
β
Ks
Ks
α
Ks
β
β
227
27
α β
2 2
2
2
Ks
Kr K1 Ks
α Ks
α Kr
α Na
β Ks
β Kr
α K1
α Ca
β Na
Ks
α K1
α Ks
β Ks
α K1
α Ca
Ks
Kr
2+
√RR− Interval
≥
≥
≥
≥ 480ms
≥
≥ 12
≤ 4
α Ks
α Kr
α Na
β Kr
β Ks
α K1
α Ca
β Na
Ks
Na
α K1
β
β
β
β
“
Ks
Ks
µ
“
µ
“
µ
µ
µ
µ
µ
µ
µ
“
µ
µ
µ µ
“
“
“
“
“
µ
µ
µ
µ
µ
β
2
2
µ µ
Ω
2 2
“
β
β Ks
Ks
Ks
“
“
“
“ “
“
β
β β
V1
V2
V3
β
V5
V6
β
I
II
III
aVL
aVR
aVF
V1
V2
V3
V4
V5
V6
“
β
“
“
V5
V6
V5
V6
β
“ “
β
“
“ “
“
β
β
V5
V6
β
2
“
µ
“
“
KCNQ1 WT
1µA
500ms
-60 -40 -20 0 20 40 60
0,0
0,5
1,0 KCNQ1 WT
Re
lative
r S
tro
m
Spannung (mV)
“
“
± µ
c.556-561del
c.1892_1893insC p.G269S
KCNQ1 WT100%
1µA
500ms
+60 mV, 3000 ms
-80 mV-60 mV
-60 -40 -20 0 20 40 60
0,0
0,5
1,0 KCNQ1 WT
p.G269S
c.1892_1893insC
c.556_561del
Rela
tiver
Str
om
Spannung (mV)
µ
+60 mV, 3000 ms
-80 mV-60 mV
p.R539L p.L273V
KCNQ1 WT100%
1µA
500ms
-60 -40 -20 0 20 40 60
0,0
0,5
1,0
1,5
2,0 KCNQ1 WT
p.L273V
p.R539L
Rela
tive
r S
trom
Spannung (mV)
µ
µ ±0, 012µ ±0, 03µ ±0, 22µ ±0, 10µ ±0, 052
µ ±0, 09
µ
c.556_561del+WT p.G269S+WTc.1892_1893insC+WT
p.R539L+WT
KCNQ1 WT100%
1µA
500msKCNQ1 WT50%
+60 mV, 3000 ms
-80mV-60 mV
p.L273V+WT
-60 -40 -20 0 20 40 60
0,0
0,5
1,0
1,5
2,0
KCNQ1 WT100%
KCNQ1 WT50%
c.556_561del+WT
p.G269S+WT
p.L273V+WT
p.R539L+WT
c.1892_1893insC+WT
Re
lativer
Str
om
Spannung (mV)
µ ±0, 03µ ±0, 06µ ±0, 21µ ±0, 13µ ±0, 04
µ ±0, 11µ ±0.05
Ks
“
µ
µ
µ
Ks
3µA
500ms
KCNQ1 WT100%+KCNE1
-80 mV-80 mV
+40 mV, 7000 ms
-40 mV
-40 -20 0 20 40
0,0
0,2
0,4
0,6
0,8
1,0 KCNQ1 WT+KCNE1
Rela
tiver
Str
om
Spannung (mV)
Ks
Ks
Ks
Ks
µ ±
Ks
Ks
Ks
µ β
µ
Ks
Ks
Ks
p.G269S+KCNE1
p.L273V+KCNE1
xKCNQ1+KCNE1
p.R539L+KCNE1
c.1892_1893insC+KCNE1
3µA
500ms
KCNQ1 WT100%+KCNE1
-80 mV-80 mV
+40 mV, 7000 ms
-40 mV
c.del556_561+KCNE1
-40 -20 0 20 40
0,0
0,2
0,4
0,6
0,8
1,0
KCNQ1 WT+KCNE1
xKCNQ1+KCNE1
p.R539L+KCNE1
p.G269S+KCNE1
c.556_561del+KCNE1
c.1892_1893insC+KCNE1
p.L273V+KCNE1
Re
lative
r S
tro
m
Spannung (mV)
Ks
Ks
β
Ks
Ks
Ks
Ks
Ks
Ks
µ Ks
µ ±0, 16µ ±0, 13µ ±0, 45µ ±0, 3µ ±0, 18
µ ±0, 52µ ±0, 20
µ ±
µ
±
“ “
Ks
“
“
Ks
Ks
Ks
-80 mV-80 mV
+40 mV, 7000 ms
-40 mV
p.L273V+KCNE1
3µA
500ms
KCNQ1 WT100%+KCNE1
-40 -20 0 20 40
-0,1
0,0
0,1
0,2
0,3
0,4
0,5
0,6
0,7
0,8
0,9
1,0
KCNQ1 WT+KCNE1
p.L273V+KCNE1
Norm
alis
iert
er
Str
om
Spannung (mV)
KCNQ1 WT+KCNE1
xKCQN1+KCNE1
c.1892_1893insC+KCNE1
p.R539L+KCNE1
p.L273V+KCNE1
p.G269S+KCNE1
c.556_561del+KCNE1
-20 -15 -10 -5 0 5 10 15
halbmaximale Aktivierung (mV)
“
“
“
Ks
Ks
±1, 5(ns)±3, 9(ns)±3, 7(∗∗)±0, 8(ns)±1, 9(ns)
±1, 3±6, 5
Ks
µ
µ
KCNQ1 WT+p.G269S+KCNE1
1µA
500ms
KCNQ1 WT+KCNE1 KCNQ1 WT50%+KCNE1 xKCNQ1+KCNE1
KCNQ1 WT+p.L273V+KCNE1 KCNQ1 WT+
c.1892_1893insC+
KCNE1
KCNQ1 WT+p.R539L+KCNE1
KCNQ1 WT+c.556_561del+
KCNE1
-80 mV-80 mV
+40 mV, 7000 ms
-40 mV
-40 -20 0 20 40
0,0
0,2
0,4
0,6
0,8
1,0
KCNQ1 WT100%+KCNE1
KCNQ1 WT50%+KCNE1
xKCNQ1+KCNE1
KCNQ1 WT+c.1892_1893insC+KCNE1
KCNQ1 WT+c.556_561del+KCNE1
KCNQ1 WT+p.G269S+KCNE1
KCNQ1 WT+p.L273V+KCNE1
KCNQ1 WT+p.R539L+KCNE1
Re
lativer
Str
om
Spannung (mV)
Ks
µ ±0, 13µ ±0, 12µ ±0, 11µ ±0, 13µ ±0, 16
µ ±0, 24µ ±0, 11µ ±0, 08
“
p.L273V+WT+KCNE1
1µA
500ms
KCNQ1 WT+KCNE1
-40 -20 0 20 40
0,0
0,1
0,2
0,3
0,4
0,5
0,6
0,7
0,8
0,9
1,0
KCNQ1 WT+KCNE1
p.L273V+WT+KCNE1
Norm
alis
iert
er
Str
om
Spannung (mV)
-80 mV-80 mV
+40 mV, 7000 ms
-40 mV
KCNQ1 WT+KCNE1
xKCQN1+KCNE1
c.1892_1893insC+WT+KCNE1
p.R539L+WT+KCNE1
p.L273V+WT+KCNE1
p.G269S+WT+KCNE1
c.556_561del+WT+KCNE1
-20 -15 -10 -5 0 5 10 15 20
halbmaximale Aktivierung (mV)
“
“
±0, 7(∗ ∗ ∗)±2, 8(∗ ∗ ∗)±3, 7(∗ ∗ ∗)±1, 7(∗ ∗ ∗)±4, 3(∗)
±1, 3±6, 5
Ks
Ks Ks
Ks
Ks
Ks
Ks
Ks
Ks
β
Ks
β
Ks
β
β
Ks
Ks
Ks
Ks
Ks
Ks
Ks
Ks
Ks
Ks
Ks
Ks
Ks
Ks
Ks
β
Ks
Ks
β
Ks
Ks
Ks
Ks
Ks
2
Ks
Ks
Ks
Ks
Ks
Ks
Ks
Ks
Ks
Ks
Ks
Ks
Ks
Ks Ks
2
2
2
2+
2
2
2
2
2
2
Ks
2
β
633 646
∆
∆
∆ Ks
Ks
Ks ∆
∆
∆
Ks
Ks
∆
Ks
∆ Ks
∆
∆
Ks
Ks ∆
Ks
≥≤
Kr
Ks
K1
+
+
µ
∆
Ks
Ks
β
Ks
Ks
Kr Ks K1
Ks
α β
Ks
Ks
Ks
Ks
Kr Ks K1
Ks α
β
Ks
Ks
