ε-Sarcoglycan, a broadly expressed homologue of the gene mutated in limb-girdle muscular dystrophy 2D
ε-Sarcoglycan, a broadly expressed homologue of the gene mutated in limb-girdle muscular dystrophy 2D
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DOI：
10.1074/jbc.272.51.32534
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发表时间：
1997-12-19
影响因子：
4.8
通讯作者：
Sanes, JR
中科院分区：
文献类型：
--
作者：
Ettinger, AJ;Feng, GP;Sanes, JR
肌聚糖是肌营养不良蛋白-糖蛋白复合体的跨膜成分，它将成年肌肉纤维中的细胞骨架与细胞外基质联系起来。已知的四种肌糖蛋白基因(阿尔法、贝塔、伽玛和德尔塔)的突变都在四肢带状肌营养不良症患者中被发现。我们已经鉴定出一种新的蛋白质，epsilon-肌聚糖，它与α-肌聚糖(Adhalin)有44%的氨基酸同源性。我们通过Northern印迹、免疫印迹和免疫荧光证明epsilon-肌聚糖是一种膜相关糖蛋白，并证明了其表达。与主要或仅在横纹肌中表达的α-三角洲肌聚糖不同，epsilon-肌聚糖广泛分布于胚胎和成人的肌肉和非肌肉细胞中。这些结果提出了在许多细胞类型中，含有肌聚糖的复合体介导膜-基质相互作用的可能性。
The sarcoglycans are transmembrane components of the dystrophin-glycoprotein complex, which links the cytoskeleton to the extracellular matrix in adult muscle fibers. Mutations in all four known sarcoglycan genes (alpha, beta, gamma, and delta) have been found in humans with limb-girdle muscular dystrophy. We have identified a novel protein, epsilon-sarcoglycan, that shares 44% amino acid identity with alpha-sarcoglycan (adhalin). We show that epsilon-sarcoglycan is a membrane-associated glycoprotein and document its expression by Northern blotting, immunoblotting, and immunofluorescence. In contrast to alpha-delta sarcoglycans, which are expressed predominantly or exclusively in striated muscle, epsilon-sarcoglycan is broadly distributed in muscle and nonmuscle cells of both embryos and adults. These results raise the possibility that sarcoglycan-containing complexes mediate membrane-matrix interactions in many cell types.