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Calpain-1 ablation partially rescues disease-associated hallmarks in models of Machado-Joseph disease
Proteolytic fragmentation of polyglutamine-expanded ataxin-3 is a concomitant and modifier of the molecular pathogenesis of Machado–Joseph disease (MJD), the most common autosomal dominant cerebellar ataxia. Calpains, a group of calcium-dependent cysteine proteases, are important mediators of ataxin...
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| Publicado no: | Hum Mol Genet |
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| Main Authors: | , , , , , , , |
| Formato: | Artigo |
| Idioma: | Inglês |
| Publicado em: |
Oxford University Press
2020
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| Assuntos: | |
| Acesso em linha: | https://ncbi.nlm.nih.gov/pmc/articles/PMC7158375/ https://ncbi.nlm.nih.gov/pubmed/31960910 https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1093/hmg/ddaa010 |
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