Author:
Huang Xuan,Lee Seungkyu,Kawaguchi Riki,Wiskow Ole,Ghosh Sulagna,Frost Devlin,Perrault Laura,Pandey Roshan,Chen Kuchuan,Klim Joseph R.,Boivin Bruno,Hermawan Crystal,Livak Kenneth J.,Geschwind Dan,Wainger Brian J.,Eggan Kevin,Bean Bruce P.,Woolf Clifford J
Abstract
AbstractThe Kv8.1 potassium ion channel encoded by the KCNV1 gene is a “silent” subunit whose biological function is unknown. In ALS patient-derived motor neurons carrying SOD1(A4V) and C9orf72 mutations, its expression is highly reduced, yielding increased vulnerability to cell death without a change in motor neuronal firing. Our data suggests that Kv8.1 modulates Kv2 channel function to impact neuronal metabolism and lipid/protein transport pathways, but not excitability.
Publisher
Cold Spring Harbor Laboratory