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Downregulation of the silent potassium channel Kv8.1 increases ALS motor neuron vulnerability

Year: 2023
Type: preprint
Abstract: The 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.
DOI: 10.1101/2023.05.07.539778
Authors: Xuan Huang, Seungkyu Lee, Riki Kawaguchi, Ole Wiskow, Sulagna Ghosh, Devlin Frost, Laura Perrault, Roshan Pandey, Kuchuan Chen, Joseph R. Klim, Bruno Boivin, Crystal Hermawan, Kenneth J. Livak, Dan Geschwind, Brian J. Wainger, Kevin Eggan, Bruce P. Bean, Clifford J Woolf
Institutions: Boston Children's Hospital, Harvard University, University of California, Los Angeles, Harvard Stem Cell Institute, Dana-Farber Cancer Institute, Massachusetts General Hospital
Language: English
Subfield: Neurology
Field: Medicine
Open Access status: green
OpenAlex ID: W4375860760
Cites: 38
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Related to: 10