Muscle Biology Unit, Department of Experimental Medical Science, Lund University, Lund, Sweden.
J Pathol. 2012 Jan;226(2):200-18. doi: 10.1002/path.3020.
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.
细胞外基质 (ECM) 通过 ECM 受体为细胞提供坚实的支架和信号。细胞-基质相互作用对于正常的生物学过程至关重要,而当它们被破坏时,可能会导致病理过程。特别是,由于 ECM-细胞膜-细胞骨架相互作用中的蛋白质突变导致的遗传性肌肉疾病的数量,突出了 ECM-细胞膜-细胞骨架相互作用在骨骼肌中的重要性。在这篇综述中,我们介绍了层粘连蛋白、胶原蛋白、dystroglycan、整合素、dystrophin 和 sarcoglycans。相应基因的突变导致各种形式的肌肉营养不良。本文还介绍了肌肉疾病的进展以及治疗方法的开发。