Mutations in KV3.3 cause the autosomal dominant neurological disorder SCA13 (spinocerebellar ataxia type 13), which leads to degeneration of the cerebellum and the spinal cord.42 The four main disease-associated mutations in KV3.3 lead to either reduced channel expression or channels with altered gating properties when expressed in Xenopus oocytes.43 The reduction in protein levels arises due to a reduction in protein half-life as SCA13 mutations generate unstable proteins that are rapidly degraded. The gene discussed is KCNC3; the disease is spinocerebellar ataxia type 13.