The extracellular matrix (ECM) provides a solid scaffold and signals to cells through ECM receptors. The cell-matrix interactions are crucial for normal biological processes and when disrupted they may lead to pathological processes. In particular, the biological importance of ECM-cell membrane-cytoskeleton interactions in skeletal muscle is accentuated by the number of inherited muscle diseases caused by mutations in proteins conferring these interactions. In this review we introduce laminins, collagens, dystroglycan, integrins, dystrophin and sarcoglycans. Mutations in corresponding genes cause various forms of muscular dystrophy. The muscle disorders are presented as well as advances toward the development of treatment. Copyright (C) 2011 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd.
机构:
IIT, Dept Biomed Engn, Chicago, IL 60616 USA
IIT, Pritzker Inst Biomed Sci & Engn, Chicago, IL 60616 USA
Edward Hines Jr VA Hosp, Dept Res, Hines, IL 60141 USAIIT, Dept Biomed Engn, Chicago, IL 60616 USA
Francis, Megan E.
Uriel, Shiri
论文数: 0引用数: 0
h-index: 0
机构:
IIT, Dept Biomed Engn, Chicago, IL 60616 USA
IIT, Pritzker Inst Biomed Sci & Engn, Chicago, IL 60616 USAIIT, Dept Biomed Engn, Chicago, IL 60616 USA
Uriel, Shiri
Brey, Eric M.
论文数: 0引用数: 0
h-index: 0
机构:
IIT, Dept Biomed Engn, Chicago, IL 60616 USA
IIT, Pritzker Inst Biomed Sci & Engn, Chicago, IL 60616 USA
Edward Hines Jr VA Hosp, Dept Res, Hines, IL 60141 USAIIT, Dept Biomed Engn, Chicago, IL 60616 USA
机构:
INSERM, Lab Osteoblast Biol & Pathol, U606, F-75475 Paris 10, France
Univ Paris 07, Hop Lariboisiere, F-75475 Paris 10, FranceINSERM, Lab Osteoblast Biol & Pathol, U606, F-75475 Paris 10, France