The Dystrophin Complex Forms a Mechanically Strong Link between the Sarcolemma and Costameric Actin

doi:10.1083/jcb.150.5.1209

Published online 4 September 2000. doi:10.1083/jcb.150.5.1209

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© The Rockefeller University Press, 0021-9525/2000//1209 $5.00
The Journal of Cell Biology, Volume 150, Number 5, , 2000 1209-1214

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The Dystrophin Complex Forms a Mechanically Strong Link between the Sarcolemma and Costameric Actin

Inna N. Rybakova^a, Jitandrakumar R. Patel^a, and James M. Ervasti^a

^a Department of Physiology, University of Wisconsin Medical School, Madison, Wisconsin 53706
Department of Physiology, 127 Service Memorial Institutes, 1300 University Avenue, Madison, WI 53706.(608) 265-5512(608) 265-3419

ervasti{at}physiology.wisc.edu

The absence of dystrophin complex leads to disorganization ofthe force-transmitting costameric cytoskeleton and disruptionof sarcolemmal membrane integrity in skeletal muscle. However,it has not been determined whether the dystrophin complex canform a mechanically strong bond with any costameric protein.We performed confocal immunofluorescence analysis of isolatedsarcolemma that were mechanically peeled from skeletal fibersof mouse hindlimb muscle. A population of ${gamma}$ -actin filaments wasstably associated with sarcolemma isolated from normal muscleand displayed a costameric pattern that precisely overlappedwith dystrophin. However, costameric actin was absent from allsarcolemma isolated from dystrophin-deficient mdx mouse muscleeven though it was localized to costameres in situ. Vinculin, ${alpha}$ -actinin, β-dystroglycan and utrophin were all retainedon mdx sarcolemma, indicating that the loss of costameric actinwas not due to generalized membrane instability. Our data demonstratethat the dystrophin complex forms a mechanically strong linkbetween the sarcolemma and the costameric cytoskeleton throughinteraction with ${gamma}$ -actin filaments. Destabilization of costamericactin filaments may also be an important precursor to the costameredisarray observed in dystrophin-deficient muscle. Finally, thesemethods will be broadly useful in assessing the mechanical integrityof the membrane cytoskeleton in dystrophic animal models lackingother costameric proteins.

Key Words: dystrophin • actin • muscular dystrophy • membrane skeleton • costameres

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