Antiprion drugs 6-aminophenanthridine and guanabenz reduce PABPN1 toxicity and aggregation in oculopharyngeal muscular dystrophy

Oculopharyngeal muscular dystrophy (OPMD) is an adult-onset syndrome characterized by progressive degeneration of specific muscles. OPMD is caused by extension of a polyalanine tract in poly(A) binding protein nuclear 1 (PABPN1). Insoluble nuclear inclusions form in diseased muscles. We have generat...

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Main Authors: Barbezier, Nicolas, Chartier, Aymeric, Bidet, Yannick, Buttstedt, Anja, Voisset, Cécile, Galons, Hervé, Blondel, Marc, Schwarz, Elisabeth, Simonelig, Martine
Format: Artigo
Jezik:Inglês
Izdano: WILEY-VCH Verlag 2011
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Research Articles
Online dostop:https://ncbi.nlm.nih.gov/pmc/articles/PMC3044817/
https://ncbi.nlm.nih.gov/pubmed/21204267
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1002/emmm.201000109
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