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Molecular discoveries of the past decade have defined many new classes of neurological disorders. Two of these, the ion channelopathies and the polyglutamine diseases, have distinct pathological mechanisms and may require different therapeutic strategies. One disorder, spinocerebellar ataxia type 6 (SCA6) is unusual: It appears to belong to both classes. Disorders of ion channel function underlie many autosomal dominant, episodic, or progressive conditions including epilepsy, periodic paralysis, migraine, and episodic ataxia.1,2⇓ Missense or truncation mutations in neurotransmitter-gated or voltage-gated ion channels disturb nerve or muscle excitability, or intracellular ionic homeostasis, and lead to neuronal dysfunction or death. The toxic effect exerted by these mutant channels occurs by a perturbing normal channel function.
Polyglutamine (polyQ) expansion disorders, such as Huntington’s disease and most autosomal dominant spinocerebellar ataxias, arise from a shared mutational mechanism,3 an abnormally expanded sequence encoding a tract of glutamine residues in any of several unrelated …
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