Differential toxicity of ataxin-3 isoforms in Drosophila models of Spinocerebellar Ataxia Type 3
Abstract
The most commonly inherited dominant ataxia, Spinocerebellar Ataxia Type 3 (SCA3), is caused by a CAG repeat expansion that encodes an abnormally long polyglutamine (polyQ) repeat in the disease protein ataxin-3, a deubiquitinase. Two major full-length isoforms of ataxin-3 exist, both of which contain the same N-terminal portion and polyQ repeat, but differ in their C-termini; one (denoted here as isoform 1) contains a motif that binds...
Paper Details
Title
Differential toxicity of ataxin-3 isoforms in Drosophila models of Spinocerebellar Ataxia Type 3
Published Date
Dec 1, 2019
Journal
Volume
132
Pages
104535 - 104535
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