KCNV1 encodes Kv8.1, a 'silent' voltage-gated potassium channel subunit that cannot form functional channels independently but modulates the activity of Kv2 channels (KCNB1/KCNB2) by shifting inactivation thresholds to more negative values and slowing inactivation rates 1. KCNV1 can also inhibit Kv3 channels and down-regulate activity of KCNC4 and KCND1 2. The gene maps to chromosome 8.3, a locus associated with benign adult familial myoclonic epilepsy 2. Clinically, KCNV1 dysfunction is implicated in amyotrophic lateral sclerosis (ALS): expression is substantially reduced in ALS patient-derived motor neurons carrying SOD1(A4V) and C9orf72 mutations 1. Loss of KCNV1 expression increases motor neuron vulnerability to cell death through dysregulated metabolism and lipid/protein transport pathways, a phenotype rescued by knocking down Kv2.2 1. Loss-of-function zebrafish models show KCNV1 mutations cause seizure-like behavior, providing evidence for its role in human epilepsy 3. KCNV1 expression is downregulated in fast ripple-generating epilepsy brain regions, suggesting involvement in seizure onset zone formation 4. Additionally, KCNV1 can be a target for HPV integration in cervical cancer 5. In cardiac tissue, KCNV1 expression increases in response to catecholamine treatment, supporting electrical function in engineered cardiac patches 6.