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一种参与肌营养不良症的黏附分子:肌营养不良聚糖结构域的结构与功能分析

An adhesion molecule involved in muscular dystrophies: structural and functional analysis of dystroglycan domains.

作者信息

Brancaccio Andrea

机构信息

CNR, Istituto di Chimica del Riconoscimento Molecolare, Istituto di Biochimica e Biochimica Clinica, Universita' Cattolica del Sacro Cuore, 00168 Rome, Italy.

出版信息

Ital J Biochem. 2003 Mar;52(1):51-4.

Abstract

Dystroglycan (DG) plays a pivotal role within the dystrophin-glycoprotein complex (DGC) which represents a major factor for muscle fibre stability upon contraction. It has been shown that many muscular dystrophy phenotypes are caused by mutations of proteins belonging to or being associated with the DGC. Due to its prominent role for muscle stability, the detailed knowledge of DG structural and functional aspects should be considered of primary importance in order to develop new treatments for neuromuscular diseases.

摘要

肌营养不良蛋白聚糖(DG)在肌营养不良蛋白-糖蛋白复合物(DGC)中起着关键作用,而DGC是肌肉纤维收缩时稳定性的主要决定因素。研究表明,许多肌肉营养不良表型是由属于DGC或与DGC相关的蛋白质突变引起的。由于DG在肌肉稳定性方面的突出作用,为了开发针对神经肌肉疾病的新疗法,对DG结构和功能方面的详细了解应被视为至关重要。

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