PABPN1 overexpression leads to upregulation of genes encoding nuclear proteins that are sequestered in oculopharyngeal muscular dystrophy nuclear inclusions

PABPN1 overexpression leads to upregulation of genes encoding nuclear proteins that are sequestered in oculopharyngeal muscular dystrophy nuclear inclusions

Oculopharyngeal muscular dystrophy (OPMD) is an adult-onset disease caused by expanded (GCN)12–17 stretches encoding the N-terminal polyalanine domain of the poly(A) binding protein nuclear 1 (PABPN1). OPMD is characterized by intranuclear inclusions (INIs) in skeletal muscle fibers, which contain P...

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Bibliographic Details
Main Authors: Louis-Philippe Corbeil-Girard, Arnaud F. Klein, A. Marie-Josée Sasseville, Hugo Lavoie, Marie-Josée Dicaire, Anik Saint-Denis, Martin Pagé, André Duranceau, François Codère, Jean-Pierre Bouchard, George Karpati, Guy A. Rouleau, Bernard Massie, Yves Langelier, Bernard Brais
Format: Article
Language:English
Published: Elsevier 2005-04-01
Series:Neurobiology of Disease
Subjects:
Muscular dystrophy
OPMD
PABPN1
Polyalanine
Intranuclear inclusion
Online Access:http://www.sciencedirect.com/science/article/pii/S0969996104002451

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http://www.sciencedirect.com/science/article/pii/S0969996104002451

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