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Comparison of spinocerebellar ataxia type 3 mouse models identifies early gain-of-function, cell-autonomous transcriptional changes in oligodendrocytes

Spinocerebellar ataxia type 3 (SCA3) is a neurodegenerative disorder caused by a polyglutamine-encoding CAG repeat expansion in the ATXN3 gene. This expansion leads to misfolding and aggregation of mutant ataxin-3 (ATXN3) and degeneration of select brain regions. A key unanswered question in SCA3 an...

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Detalhes bibliográficos
Publicado no:Hum Mol Genet
Main Authors: Ramani, Biswarathan, Panwar, Bharat, Moore, Lauren R., Wang, Bo, Huang, Rogerio, Guan, Yuanfang, Paulson, Henry L.
Formato: Artigo
Idioma:Inglês
Publicado em: Oxford University Press 2017
Assuntos:
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC5886175/
https://ncbi.nlm.nih.gov/pubmed/28854700
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1093/hmg/ddx224
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