What Is the Role of Potassium Channels in Ataxia?

There is emerging evidence from experimental models that different types of potassium channels have a major role in shaping the electrophysiologic properties of Purkinje cells. Studies in mouse models of spinocerebellar ataxias (SCAs) indicate that altered Purkinje cell firing and excessive accumulation of intracellular calcium are 2 major mechanisms of cerebellar ataxia.1-6 Potassium channel mutations are an increasingly recognized cause of dominantly inherited SCA7-11 and can also manifest with episodic ataxia.12 These findings indicate that potassium channels provide a potential therapeutic target in these disorders.

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