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J Pathol. 2012 Jan;226(2):200-18. doi: 10.1002/path.3020.

Cell-matrix interactions in muscle disease.

Author information

1
Muscle Biology Unit, Department of Experimental Medical Science, Lund University, Lund, Sweden.

Abstract

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.

PMID:
21989954
DOI:
10.1002/path.3020
[Indexed for MEDLINE]

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