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The autophagy/lysosome pathway is impaired in SCA7 patients and SCA7 knock-in mice

Archive ouverte | Alves, Sandro | CCSD

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Lentiviral vector-mediated overexpression of mutant ataxin-7 recapitulates SCA7 pathology and promotes accumulation of the FUS/TLS and MBNL1 RNA-binding proteins

Archive ouverte | Alves, Sandro | CCSD

International audience. Background: We used lentiviral vectors (LVs) to generate a new SCA7 animal model overexpressing a truncated mutant ataxin-7 (MUT ATXN7) fragment in the mouse cerebellum, in order to character...

PML clastosomes prevent nuclear accumulation of mutant ataxin-7 and other polyglutamine proteins.

Archive ouverte | Janer, Alexandre | CCSD

International audience. The pathogenesis of spinocerebellar ataxia type 7 and other neurodegenerative polyglutamine (polyQ) disorders correlates with the aberrant accumulation of toxic polyQ-expanded proteins in the...

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