Ohlendieck K, Swandulla D
Muscle Biology Laboratory, Department of Biology, Maynooth University, National University of Ireland, Maynooth, Co. Kildare, Irland.
Institut für Physiologie 2, Rheinische Friedrich-Wilhelms-Universität Bonn, Bonn, Deutschland.
Pathologe. 2017 Feb;38(1):21-29. doi: 10.1007/s00292-017-0265-1.
Progressive myofibrosis plays a key role in Duchenne muscular dystrophy. The dystrophic loss of contractile cells triggers a relatively nonspecific restructuring of the surrounding mesenchyme. The increase in connective and fatty tissue leads to muscular weakness and is therefore of critical importance for the cellular pathogenesis of muscular dystrophy. The systematic biochemical analysis of fibrosis using comparative proteomics has identified a number of extracellular matrix proteins that are indirectly involved in muscular dystrophy. An increased concentration was established for collagen I, collagen IV, collagen VI, periostin, dermatopontin, fibronectin, biglycan, asporin, decorin, prolargin, mimecan and lumican. Based on these findings, the identified matrix proteins can now be characterized biochemically and their exact pathophysiological role in Duchenne muscular dystrophy determined.
进行性肌纤维化在杜兴氏肌营养不良症中起关键作用。收缩性细胞的营养不良性丧失引发周围间充质的相对非特异性重构。结缔组织和脂肪组织的增加导致肌肉无力，因此对肌营养不良症的细胞发病机制至关重要。使用比较蛋白质组学对纤维化进行系统的生化分析，已鉴定出一些间接参与肌营养不良症的细胞外基质蛋白。已确定I型胶原、IV型胶原、VI型胶原、骨膜蛋白、皮肤桥蛋白、纤连蛋白、双糖链蛋白聚糖、抑瘤素、核心蛋白聚糖、前解聚蛋白、 mimecan和光蛋白聚糖的浓度增加。基于这些发现，现已对鉴定出的基质蛋白进行生化表征，并确定它们在杜兴氏肌营养不良症中的确切病理生理作用。
