Rbfox1 Downregulation and Altered Calpain 3 Splicing by FRG1 in a Mouse Model of Facioscapulohumeral Muscular Dystrophy (FSHD)

Facioscapulohumeral muscular dystrophy (FSHD) is a common muscle disease whose molecular pathogenesis remains largely unknown. Over-expression of FSHD region gene 1 (FRG1) in mice, frogs, and worms perturbs muscle development and causes FSHD–like phenotypes. FRG1 has been implicated in splicing, and...

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Bibliographic Details
Main Authors: Pistoni, Mariaelena, Shiue, Lily, Cline, Melissa S., Bortolanza, Sergia, Neguembor, Maria Victoria, Xynos, Alexandros, Ares, Manuel, Gabellini, Davide
Format: Online
Language:English
Published: Public Library of Science 2013
Online Access:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3536703/