Finding the sweet spot: assembly and glycosylation of the dystrophin-associated glycoprotein complex.

Finding the sweet spot: assembly and glycosylation of the dystrophin-associated glycoprotein complex.
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DOI:
10.1002/ar.22974
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发表时间:
2014-09
影响因子:
2
通讯作者:
Townsend, Dewayne
Townsend, Dewayne
中科院分区:
医学4区
文献类型:
--
作者:
Townsend, Dewayne

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肌营养不良蛋白相关糖蛋白复合物(DGC)是一组糖蛋白,对横纹肌和许多其他组织的正常功能至关重要。最近的遗传研究表明,这种复合物的成分与十几种形式的肌肉萎缩症有关。此外,DGC的破坏与许多形式的获得性疾病有关。这篇综述的目的是总结关于肌营养不良蛋白相关蛋白的加工和组装的知识现状,主要集中在肌营养不良聚糖异二聚体和肌聚糖复合物上。这些蛋白质形成DGC的跨膜部分,并经历复杂的多步骤加工,包括蛋白水解裂解、差异组装以及N和o糖基化。负责这一过程的酶和描述这些事件的序列和亚细胞定位的模型进行了讨论。
The dystrophin-associated glycoprotein complex (DGC) is a collection of glycoproteins that are essential for the normal function of striated muscle and many other tissues. Recent genetic studies have implicated the components of this complex in over a dozen forms of muscular dystrophy. Furthermore, disruption of the DGC has been implicated in many forms of acquired disease. This review aims to summarize the current state of knowledge regarding the processing and assembly of dystrophin associated proteins with a focus primarily on the dystroglycan heterodimer and the sarcoglycan complex. These proteins form the transmembrane portion of the DGC and undergo a complex multi-step processing with proteolytic cleavage, differential assembly, and both N- and O-glycosylation. The enzymes responsible for this processing and a model describing the sequence and subcellular localization of these events are discussed.
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