The Truncated C-terminal Fragment of Mutant ATXN3 Disrupts Mitochondria Dynamics in Spinocerebellar Ataxia Type 3 Models

The Truncated C-terminal Fragment of Mutant ATXN3 Disrupts Mitochondria Dynamics in Spinocerebellar Ataxia Type 3 Models

Spinocerebellar ataxia type 3 (SCA3), known as Machado-Joseph disease, is an autosomal dominant disease caused by an abnormal expansion of polyglutamine in ATXN3 gene, leading to neurodegeneration in SCA3 patients. Similar to other neurodegenerative diseases, the dysfunction of mitochondria is obser...

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Bibliographic Details
Main Authors: Jung-Yu Hsu, Yu-Ling Jhang, Pei-Hsun Cheng, Yu-Fan Chang, Su-Han Mao, Han-In Yang, Chia-Wei Lin, Chuan-Mu Chen, Shang-Hsun Yang
Format: Article
Language:English
Published: Frontiers Media S.A. 2017-06-01
Series:Frontiers in Molecular Neuroscience
Subjects:
truncated aTXN3
spinocerebellar ataxia type 3
mitochondria dynamics
transgenic mice
neurodegeneration
fusion and fission
Online Access:http://journal.frontiersin.org/article/10.3389/fnmol.2017.00196/full

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