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This Article Full Text Full Text (PDF) Supplemental Figure All Versions of this Article: 294/5/C1175    most recent 00049.2008v1 Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Google Scholar Articles by Zhang, J. Articles by Chen, J. PubMed PubMed Citation Articles by Zhang, J. Articles by Chen, J.

MUSCLE CELL BIOLOGY AND CELL MOTILITY

Syncoilin is required for generating maximum isometric stress in skeletal muscle but dispensable for muscle cytoarchitecture

¹Department of Medicine, University of California, San Diego, La Jolla; ²Department of Orthopaedic Surgery and Bioengineering, University of California, San Diego and Veterans Affairs Medical Centers, San Diego; ³Skaggs School of Pharmacy and Pharmaceutical Sciences, University of California, San Diego, La Jolla, California; ⁴State Key Laboratory of Virology, Wuhan University, Wuhan, People's Republic of China; and ⁵Istituto di Ricovero e Cura a Carattere Scientifico, Scientific and Technology Pole, Multimedica, Milan, Italy

Submitted 31 January 2008 ; accepted in final form 18 March 2008

Syncoilin is a striated muscle-specific intermediate filament-like protein, which is part of the dystrophin-associated protein complex (DPC) at the sarcolemma and provides a link between the extracellular matrix and the cytoskeleton through its interaction with -dystrobrevin and desmin. Its upregulation in various neuromuscular diseases suggests that syncoilin may play a role in human myopathies. To study the functional role of syncoilin in cardiac and skeletal muscle in vivo, we generated syncoilin-deficient (syncoilin^–/–) mice. Our detailed analysis of these mice up to 2 yr of age revealed that syncoilin is entirely dispensable for cardiac and skeletal muscle development and maintenance of cellular structure but is required for efficient lateral force transmission during skeletal muscle contraction. Notably, syncoilin^–/– skeletal muscle generates less maximal isometric stress than wild-type (WT) muscle but is as equally susceptible to eccentric contraction-induced injury as WT muscle. This suggests that syncoilin may play a supportive role for desmin in the efficient coupling of mechanical stress between the myofibril and fiber exterior. It is possible that the reduction in isometric stress production may predispose the syncoilin skeletal muscle to a dystrophic condition.

intermediate filament; sarcomere; mutant mouse