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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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Detalles Bibliográficos
Autores principales: 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
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5476786/
https://www.ncbi.nlm.nih.gov/pubmed/28676741
http://dx.doi.org/10.3389/fnmol.2017.00196