Differential toxicity of ataxin-3 isoforms in Drosophila models of Spinocerebellar Ataxia Type 3

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 conta...

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Bibliographic Details

Published in:	Neurobiology of Disease
Main Authors:	Sean L. Johnson, Jessica R. Blount, Kozeta Libohova, Bedri Ranxhi, Henry L. Paulson, Wei-Ling Tsou, Sokol V. Todi
Format:	Article
Language:	English
Published:	Elsevier 2019-12-01
Subjects:	Ataxia Drosophila Isoform Neurodegeneration Polyglutamine Proteasome
Online Access:	http://www.sciencedirect.com/science/article/pii/S0969996119302037