title: Cell-matrix interactions in muscle disease.
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.
authors: Muscle Biology Unit, Department of Experimental Medical Science, Lund University, Lund, Sweden.
authors: Virginie
authors: V
authors: Carmignac
authors: Madeleine
authors: M
authors: Durbeej
mesh_terms: D000818:Animals; D002450:Cell Communication / Q000502:physiology*; D024141:Collagen Type IV / Q000737:chemistry / Q000502:physiology; D004195:Disease Models, Animal; D049030:Dystroglycans / Q000737:chemistry / Q000502:physiology; D016189:Dystrophin / Q000737:chemistry / Q000502:physiology; D005109:Extracellular Matrix / Q000473:pathology* / Q000502:physiology; D016326:Extracellular Matrix Proteins / Q000737:chemistry / Q000502:physiology*; D006801:Humans; D016023:Integrins / Q000737:chemistry / Q000502:physiology; D007797:Laminin / Q000737:chemistry / Q000502:physiology; D009136:Muscular Dystrophies / Q000473:pathology*; D049031:Sarcoglycans / Q000737:chemistry / Q000502:physiology
chemical_list: D024141:Collagen Type IV; D016189:Dystrophin; D016326:Extracellular Matrix Proteins; D016023:Integrins; D007797:Laminin; D049031:Sarcoglycans; D049030:Dystroglycans