The spinocerebellar ataxia type 1 protein, ataxin-1, has RNA-binding activity that is inversely affected by the length of its polyglutamine tract

Shinji Yue, Heliane G. Serra, Huda Y. Zoghbi, Harry T Orr

Research output: Contribution to journalArticlepeer-review

123 Scopus citations

Abstract

Spinocerebellar ataxia type 1 (SCA1) is an autosomal dominant neurodegenerative disease caused by the expansion of a polyglutamine tract within the SCA1 product, ataxin-1. Previously, using transgenic mice, it was demonstrated that in order for a mutant allele of ataxin-1 to cause disease it must be transported to the nucleus of the neuron. Using an in vitro RNA-binding assay, we demonstrate that ataxin-1 does bind RNA and that this binding diminishes as the length of its polyglutamine tract increases. These observations suggest that ataxin-1 plays a role in RNA metabolism and that the expansion of the polyglutamine tract may alter this function.

Original languageEnglish (US)
Pages (from-to)25-30
Number of pages6
JournalHuman molecular genetics
Volume10
Issue number1
DOIs
StatePublished - Jan 1 2001

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