Deregulation of the ubiquitin-proteasome system is the predominant molecular pathology in OPMD animal models and patients

Deregulation of the ubiquitin-proteasome system is the predominant molecular pathology in OPMD animal models and patients

<p>Abstract</p> <p>Oculopharyngeal muscular dystrophy (OPMD) is a late-onset progressive muscle disorder caused by a poly-alanine expansion mutation in the Poly(A) Binding Protein Nuclear 1 (PABPN1). The molecular mechanisms that regulate disease onset and progression are largely u...

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Main Authors: Anvar Seyed, 't Hoen Peter AC, Venema Andrea, van der Sluijs Barbara, van Engelen Baziel, Snoeck Marc, Vissing John, Trollet Capucine, Dickson George, Chartier Aymeric, Simonelig Martine, van Ommen Gert-Jan B, van der Maarel Silvere M, Raz Vered
Format: Article
Language:English
Published: BMC 2011-04-01
Series:Skeletal Muscle
Online Access:http://www.skeletalmusclejournal.com/content/1/1/15

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http://www.skeletalmusclejournal.com/content/1/1/15

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