Broad Influence of Mutant Ataxin-3 on the Proteome of the Adult Brain, Young Neurons, and Axons Reveals Central Molecular Processes and Biomarkers in SCA3/MJD Using Knock-In Mouse Model

Broad Influence of Mutant Ataxin-3 on the Proteome of the Adult Brain, Young Neurons, and Axons Reveals Central Molecular Processes and Biomarkers in SCA3/MJD Using Knock-In Mouse Model

Spinocerebellar ataxia type 3 (SCA3/MJD) is caused by CAG expansion mutation resulting in a long polyQ domain in mutant ataxin-3. The mutant protein is a special type of protease, deubiquitinase, which may indicate its prominent impact on the regulation of cellular proteins levels and activity. Yet,...

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Bibliographic Details
Main Authors: Kalina Wiatr, Łukasz Marczak, Jean-Baptiste Pérot, Emmanuel Brouillet, Julien Flament, Maciej Figiel
Format: Article
Language:English
Published: Frontiers Media S.A. 2021-06-01
Series:Frontiers in Molecular Neuroscience
Subjects:
spinocerebellar ataxia type 3 (SCA3)
Machado-Joseph disease (MJD)
ataxin-3
neurodegenerative
proteome
axon
Online Access:https://www.frontiersin.org/articles/10.3389/fnmol.2021.658339/full

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