Dystroglycan in development and disease
Dystroglycan in development and disease
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DOI：
10.1016/s0955-0674(98)80034-3
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发表时间：
1998-10-01
影响因子：
7.5
通讯作者：
Campbell, KP
中科院分区：
文献类型：
--
作者：
Durbeej, M;Henry, MD;Campbell, KP
在过去的两年里，我们对肌营养不良蛋白聚糖（一种细胞表面层粘连蛋白/聚集蛋白受体）的结构和功能的理解急剧增加。结构研究，分析其结合伙伴，并有针对性的基因破坏都有助于阐明的生物学作用的肌营养不良蛋白聚糖在发展和疾病。现在很明显，肌营养不良蛋白聚糖在几种肌营养不良症的发病机制中起着关键作用，并作为人类病原体的受体，以及参与早期发育，器官形态发生和突触发生。
Our understanding of the structure and function of dystroglycan, a cell surface laminin/agrin receptor, has increased dramatically over the past two years. Structural studies, analysis of its binding partners, and targeted gene disruption have all contributed to the elucidation of the biological role of dystroglycan in development and disease. It is now apparent that dystroglycan plays a critical role in the pathogenesis of several muscular dystrophies and serves as a receptor for a human pathogen as well as being involved in early development, organ morphogenesis, and synaptogenesis.