Embryonic expression patterns of the Drosophila dystrophin-associated glycoprotein complex orthologs
Embryonic expression patterns of the Drosophila dystrophin-associated glycoprotein complex orthologs
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DOI：
10.1016/j.modgep.2003.09.004
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发表时间：
2004-03-01
影响因子：
1.2
通讯作者：
Noordermeer, JN
中科院分区：
文献类型：
--
作者：
Dekkers, LC;van der Plas, MC;Noordermeer, JN
编码人肌营养不良蛋白相关糖蛋白复合物（DGC）的蛋白质的基因突变导致杜氏肌营养不良症、贝克尔肌营养不良症和肢带肌营养不良症。DGC蛋白的亚组形成组织特异性复合物，其被认为在肌肉和神经肌肉接头中发挥结构和信号传导作用。此外，导致杜氏肌营养不良症的肌营养不良蛋白基因突变通常与认知和行为缺陷相关，表明肌营养不良蛋白在神经系统中的作用。尽管在过去十年中取得了重大进展，但关于肌营养不良蛋白和其他DGC蛋白在肌肉和外周及中枢神经系统中所起作用的许多基本问题仍有待回答。DGC基因功能的哺乳动物模型由于存在完全或部分冗余的基因而变得复杂，这些基因的功能可以掩盖给定DGC基因失活的影响。果蝇的基因组编码大多数哺乳动物DGC蛋白亚类的单一直系同源物，从而可能简化其功能分析。我们在这里报告的胚胎mRNA表达模式的个别DGC直系同源。我们发现它们主要在神经系统和肌肉中表达。肌营养不良蛋白、肌营养不良蛋白样蛋白、肌营养不良蛋白聚糖样蛋白、肌营养蛋白样蛋白1和所有三种肌聚糖直系同源物都存在于大脑和腹神经索中，而肌营养不良蛋白、肌营养不良蛋白样蛋白、肌营养不良蛋白样蛋白、肌营养蛋白样蛋白2、肌聚糖α和肌聚糖δ在中胚层来源的组织（即体壁和肠道周围的肌肉）的不同且有时重叠的结构域中表达。(C)2003 Elsevier B.V.保留所有权利。
Mutations in genes encoding proteins of the human dystrophin-associated glycoprotein complex (DGC) cause the Duchenne, Becker and limb-girdle muscular dystrophies. Subsets of the DGC proteins form tissue-specific complexes which are thought to play structural and signaling roles in the muscle and at the neuromuscular junction. Furthermore, mutations in the dystrophin gene that lead to Duchenne muscular dystrophy are frequently associated with cognitive and behavioral deficits, suggesting a role for dystrophin in the nervous system. Despite significant progress over the past decade, many fundamental questions about the roles played by dystrophin and the other DGC proteins in the muscle and peripheral and central nervous systems remain to be answered. Mammalian models of DGC gene function are complicated by the existence of fully or partially redundant genes whose functions can mask effects of the inactivation of a given DGC gene. The genome of the fruitfly Drosophila melanogaster encodes a single ortholog of the majority of the mammalian DGC protein subclasses, thus potentially simplifying their functional analysis. We report here the embryonic mRNA expression patterns of the individual DGC orthologs. We find that they are predominantly expressed in the nervous system and in muscle. Dystrophin, dystrobrevin-like, dystroglycan-like, syntrophin-like 1, and all three sarcoglycan orthologs are found in the brain and the ventral nerve cord, while dystrophin, dystrobrevin-like, dystroglycan-like, syntrophin-like 2, sarcoglycan alpha and sarcoglycan delta are expressed in distinct and sometimes overlapping domains of mesoderm-derived tissues, i.e. muscles of the body wall and around the gut. (C) 2003 Elsevier B.V. All rights reserved.