Muscular dystrophy-associated SUN1 and SUN2 variants disrupt nuclear-cytoskeletal connections and myonuclear organization.

Proteins of the nuclear envelope (NE) are associated with a range of inherited disorders, most commonly involving muscular dystrophy and cardiomyopathy, as exemplified by Emery-Dreifuss muscular dystrophy (EDMD). EDMD is both genetically and phenotypically variable, and some evidence of modifier gen...

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Main Authors: Peter Meinke, Elisabetta Mattioli, Farhana Haque, Susumu Antoku, Marta Columbaro, Kees R Straatman, Howard J Worman, Gregg G Gundersen, Giovanna Lattanzi, Manfred Wehnert, Sue Shackleton
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2014-09-01
Series:PLoS Genetics
Online Access:http://europepmc.org/articles/PMC4161305?pdf=render