Schofield J N, Górecki D C, Blake D J, Davies K, Edwards Y H
MRC Human Biochemical Genetics Department, London, United Kingdom.
Dev Dyn. 1995 Oct;204(2):178-85. doi: 10.1002/aja.1002040208.
Alpha dystroglycan (156 kDa DAG) and beta dystroglycan (43 kDa DAG) are encoded by the same gene and are components of the dystrophin-associated membrane glycoprotein complex. The dystroglycans together with dystrophin form a link between the extracellular matrix and the intracellular cytoskeleton of the muscle fibre. Using in situ hybridisation to mRNA in embryo sections we have examined the expression of the mouse dystroglycan gene. Dystroglycan transcripts are ubiquitously expressed throughout development but are most abundant in cardiac, skeletal and smooth muscle and in ependymal cells lining the developing neural tube and brain. The expression patterns of dystroglycan and dystrophin overlap in major muscle systems during development, suggesting that the dystrophin-dystroglycan complex plays an important role during myogenesis. In contrast, the major sites of utrophin expression do not co-localize with those of dystroglycan suggesting that utrophin may interact with a distinct membrane-associated complex in these non-muscle sites. In mdx embryos the pattern of distribution of dystroglycan mRNA remains unchanged, as do those of utrophin and apo-dystrophin mRNAs. This observation implies that the observed changes in the relative abundance of DAGs and utrophin in dystrophin-deficient muscle occur post-transcriptionally.
α-肌营养不良聚糖(156 kDa DAG)和β-肌营养不良聚糖(43 kDa DAG)由同一基因编码,是肌营养不良蛋白相关膜糖蛋白复合物的组成成分。肌营养不良聚糖与肌营养不良蛋白共同在细胞外基质和肌纤维的细胞内细胞骨架之间形成连接。利用胚胎切片mRNA原位杂交技术,我们检测了小鼠肌营养不良聚糖基因的表达情况。肌营养不良聚糖转录本在整个发育过程中普遍表达,但在心脏、骨骼肌和平滑肌以及发育中的神经管和脑内衬的室管膜细胞中最为丰富。在发育过程中,肌营养不良聚糖和肌营养不良蛋白的表达模式在主要肌肉系统中重叠,这表明肌营养不良蛋白-肌营养不良聚糖复合物在肌发生过程中起重要作用。相比之下,抗肌萎缩蛋白聚糖的主要表达位点与肌营养不良聚糖的主要表达位点并不共定位,这表明抗肌萎缩蛋白聚糖可能在这些非肌肉位点与一种不同的膜相关复合物相互作用。在mdx胚胎中,肌营养不良聚糖mRNA的分布模式保持不变,抗肌萎缩蛋白聚糖和脱辅基肌营养不良蛋白mRNA的分布模式也保持不变。这一观察结果表明,在肌营养不良蛋白缺陷的肌肉中观察到的DAGs和抗肌萎缩蛋白聚糖相对丰度的变化发生在转录后水平。