Browse by author
Lookup NU author(s): Dr Lars Klinge, Dr Steven Laval, Sharon Foster, Faye Haldane, Professor Volker StraubORCiD, Dr Rita Barresi, Emerita Professor Katherine Bushby
Full text for this publication is not currently held within this repository. Alternative links are provided below where available.
The dysferlin gene is mutated in limb-girdle muscular dystrophy type 2B, Miyoshi myopathy, and distal anterior compartment myopathy. In mature skeletal muscle, dysferlin is located predominantly at the sarcolemma, where it plays a role in membrane fusion and repair. To investigate the role of dysferlin during early muscle differentiation, its localization was studied at high resolution in a muscle cell line. This demonstrated that dysferlin is not expressed at the plasmalemma of myotubes but mostly localizes to the T-tubule network. However, dysferlin translocated to the site of injury and toward the plasma membrane in a Ca2+-dependent fashion in response to a newly designed in vitro wounding assay. This reaction was specific to the full-length protein, as heterologously expressed deletion mutants of distinct C2 domains of dysferlin did not show this response. These results shed light on the dynamics of muscle membrane repair and are highly indicative of a specific role of dysferlin in this process in early myogenesis. © FASEB.
Author(s): Klinge L, Laval S, Keers S, Haldane F, Straub V, Barresi R, Bushby K
Publication type: Article
Publication status: Published
Journal: FASEB Journal
Year: 2007
Volume: 21
Issue: 8
Pages: 1768-1776
ISSN (print): 0892-6638
ISSN (electronic): 1530-6860
Publisher: Federation of American Societies for Experimental Biology
URL: http://dx.doi.org/10.1096/fj.06-7659com
DOI: 10.1096/fj.06-7659com
PubMed id: 17363620
Altmetrics provided by Altmetric
