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Preventing Ataxin-3 protein cleavage mitigates degeneration in a Drosophila model of SCA3

Protein cleavage is a common feature in human neurodegenerative disease. Ataxin-3 protein with an expanded polyglutamine (polyQ) repeat causes spinocerebellar ataxia type-3 (SCA3), also called Machado–Joseph disease, and is cleaved in mammalian cells, transgenic mice and SCA3 patient brain tissue. H...

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Autors principals: Jung, Joonil, Xu, Kexiang, Lessing, Derek, Bonini, Nancy M.
Format: Artigo
Idioma:Inglês
Publicat: Oxford University Press 2009
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Accés en línia:https://ncbi.nlm.nih.gov/pmc/articles/PMC2778376/
https://ncbi.nlm.nih.gov/pubmed/19783548
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1093/hmg/ddp456
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