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First published online November 7, 2007

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Keratin shows its muscle

The strength and integrity of striated muscle cells depends crucially on links between the sarcolemma (the plasma membrane of muscle cells) and the underlying myofibrils (which orchestrate muscle contraction). The sarcolemma is organised into structures called costameres, which are linked to the contractile apparatus by several different cytoplasmic filaments, including intermediate filaments. On p. 3999, Robert Bloch and colleagues investigate the roles of the keratin component of these intermediate filaments by looking closely at the sarcolemma and costameres of keratin-19-null mice. These mice have mild myopathy; the link between the sarcolemma is disrupted, and there is a large gap between the sarcolemma and adjacent myofibrils, which becomes filled with mitochondria. Loss of keratin 19 also compromises costamere organisation, affecting in particular the cytoskeletal binding partners of the important dystrophin-dystroglycan complex. The authors propose that the myopathic phenotype of these mice is due in part to a shift in distribution of mitochondria – because subsarcolemmal mitochondria do not function as efficiently as intermyofibrillar mitochondria. They conclude that keratin 19 is important for organising costameres and their links to the contractile apparatus, and that it controls the distribution of mitochondria in skeletal muscle fibres.

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Related articles in JCS:

Absence of keratin 19 in mice causes skeletal myopathy with mitochondrial and sarcolemmal reorganization

Michele R. Stone, Andrea O'Neill, Richard M. Lovering, John Strong, Wendy G. Resneck, Patrick W. Reed, Diana M. Toivola, Jeanine A. Ursitti, M. Bishr Omary, and Robert J. Bloch
JCS 2007 120: 3999-4008. [Abstract] [Full Text]  

This Article Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Related articles in JCS Similar articles in this journal Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via Google Scholar Google Scholar Search for Related Content Social Bookmarking                         What's this?