Assessment of Purkinje neuron degeneration in the flocculus vs. medial cerebellum in a mouse model of spinocerebellar ataxia type 13 (SCA13)
Anna Lena Langen, Oskar Markkula, Thanh Le, Rashmitha Senthilvel Selvakumar, Ian D. Forsythe, Conny Kopp-Scheinpflug · FENS Forum 2024
Aims: SCA13 is a hereditary disease caused by an autosomal-dominant mutation in the KCNC3 gene which encodes the Kv3.3 potassium channel subunit of high-voltage gated potassium channels. In humans, SCA13 leads to late onset ataxia, cerebellar atrophy, and the R420H mutation also causes sound localization deficits. Kv3.3 is abundantly expressed in the cerebellum where it promotes fast repolarization of action potentials and enables Purkinje neurons (PN) to fire bursts of spikes at very high rates. Here, we compared differential degrees of PN degeneration in the flocculus and the medial cerebell
ePoster · Neuroscience