Ataxin-1 Regulators in the Spotlight

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Science  04 Jul 2003:
Vol. 301, Issue 5629, pp. 59-60
DOI: 10.1126/science.1086311

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Triplet repeat diseases are characterized by expansions of the polyglutamine tracts of specific proteins, but exactly how these expansions contribute to disease pathogenesis is still under debate. In his Perspective, Heintz discusses new results (published elsewhere) showing that the polyglutamine tract of ataxin-1 (the protein mutated in spinocerebellar ataxia 1) is not sufficient to cause pathogenesis but does promote the binding of regulatory proteins that increase the stability and accumulation of the mutant ataxin-1.