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From T-tubule to sarcolemma: Damage-induced dysferlin translocation in early myogenesis

Lookup NU author(s): Dr Lars Klinge, Dr Steven Laval, Sharon Foster, Faye Haldane, Professor Volker Straub, Dr Rita Barresi, Emerita Professor Katherine Bushby

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Abstract

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.


Publication metadata

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


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