Human Molecular Genetics, Vol 6, 513-518, Copyright © 1997 by Oxford University Press
EN Burright, JD Davidson, LA Duvick, B Koshy, HY Zoghbi and HT Orr
Spinocerebellar ataxia type 1 (SCA1) is an autosomal dominant
neurodegenerative disorder caused by the expansion of a polyglutamine tract
within the SCA1 gene product, ataxin-1. Expansion of this tract is believed
to result in a gain of function by the mutant protein, perhaps through
altered self-associations or interactions with other cellular proteins. We
have used the yeast two hybrid system to determine if ataxin-1 is capable
of multimerization. This analysis revealed that ataxin-1 does have the
ability to self-associate, however, this association does not appear to be
influenced by expansion of the polyglutamine tract. Consistent with this
finding, deletion analysis excluded the involvement of the polyglutamine
tract in ataxin- 1 self-association, and instead localized the
multimerization region to amino acids 495-605 of the wild type protein.
These results, while identifying an ataxin-1 self-interaction region, fail
to support a proposed model of polar-zipper mediated multimerization
involving the ataxin-1 polyglutamine tract.
ARTICLES
Identification of a self-association region within the SCA1 gene product, ataxin-1
Department of Laboratory Medicine and Pathology, University of Minnesota, Minneapolis 55455, USA.
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