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Article

Sirtuin inhibition protects from the polyalanine muscular dystrophy protein PABPN1

7 Apr 2008

Abstract excerpt

Oculopharyngeal muscular dystrophy (OPMD) is caused by polyalanine expansion in nuclear protein PABPN1 [poly(A) binding protein nuclear 1] and characterized by muscle degeneration. Druggable modifiers of proteotoxicity in degenerative diseases, notably the longevity modulators sirtuins, may constitute useful therapeutic targets. However, the modifiers of mutant PABPN1 are unknown. Here, we report that longevity...

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PMID: 18397876
DOI: 10.1093/hmg/ddn109

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