From: First de novo KCND3 mutation causes severe Kv4.3 channel dysfunction leading to early onset cerebellar ataxia, intellectual disability, oral apraxia and epilepsy
Kv4.3
Current density at +83 mV (pA)
Activation
Inactivation
V1/2 (mV)
Slope (mV)
WT (n = 8)
8561 ± 620
−13.3 ± 3.2
12.7 ± 0.8
−59.7 ± 6.1
4.7 ± 0.5
dupRVF (n = 9)
3861 ± 668**
46.0 ± 2.1**
11.7 ± 0.5
2.3 ± 7.0**
15.3 ± 2.0*