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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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Udgivet i:Front Mol Neurosci
Main Authors: Hsu, Jung-Yu, Jhang, Yu-Ling, Cheng, Pei-Hsun, Chang, Yu-Fan, Mao, Su-Han, Yang, Han-In, Lin, Chia-Wei, Chen, Chuan-Mu, Yang, Shang-Hsun
Format: Artigo
Sprog:Inglês
Udgivet: Frontiers Media S.A. 2017
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Online adgang:https://ncbi.nlm.nih.gov/pmc/articles/PMC5476786/
https://ncbi.nlm.nih.gov/pubmed/28676741
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.3389/fnmol.2017.00196
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