Transgenic expression of an expanded (GCG)13 repeat PABPN1 leads to weakness and coordination defects in mice

Transgenic expression of an expanded (GCG)13 repeat PABPN1 leads to weakness and coordination defects in mice

Oculopharyngeal muscular dystrophy (OPMD) is a late-onset disorder caused by a (GCG)n trinucleotide repeat expansion in the poly(A) binding protein nuclear-1 (PABPN1) gene, which in turn leads to an expanded polyalanine tract in the protein. We generated transgenic mice expressing either the wild ty...

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Bibliographic Details
Main Authors: Patrick Dion, Vijayalakshmi Shanmugam, Claudia Gaspar, Christiane Messaed, Inge Meijer, André Toulouse, Janet Laganiere, Julie Roussel, Daniel Rochefort, Simon Laganiere, Carol Allen, George Karpati, Jean-Pierre Bouchard, Bernard Brais, Guy A. Rouleau
Format: Article
Language:English
Published: Elsevier 2005-04-01
Series:Neurobiology of Disease
Subjects:
OPMD
Dystrophy
CNS
Transgenic mice
PABPN1
Nuclear inclusions
Online Access:http://www.sciencedirect.com/science/article/pii/S096999610400227X

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http://www.sciencedirect.com/science/article/pii/S096999610400227X

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