O-甘露糖基化途径：糖基转移酶和与先天性肌营养不良症相关的蛋白。
The o-mannosylation pathway: glycosyltransferases and proteins implicated in congenital muscular dystrophy.
机构信息
Complex Carbohydrate Research Center and Department of Biochemistry and Molecular Biology, University of Georgia, Athens, Georgia 30602, USA.
出版信息
J Biol Chem. 2013 Mar 8;288(10):6930-5. doi: 10.1074/jbc.R112.438978. Epub 2013 Jan 17.
Abstract
Several forms of congenital muscular dystrophy, referred to as dystroglycanopathies, result from defects in the protein O-mannosylation biosynthetic pathway. In this minireview, I discuss 12 proteins involved in the pathway and how they play a role in the building of glycan structures (most notably on the protein α-dystroglycan) that allow for binding to multiple proteins of the extracellular matrix.
摘要
几种先天性肌肉萎缩症，称为 dystroglycanopathies，是由于蛋白 O-甘露糖基化生物合成途径的缺陷引起的。在这篇小综述中，我将讨论该途径涉及的 12 种蛋白以及它们在聚糖结构（尤其是在蛋白 α- dystroglycan 上）形成中所起的作用，这些聚糖结构允许与细胞外基质的多种蛋白结合。
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