Duchenne muscular dystrophy (DMD) is a genetic disease caused by mutations in the dystrophin gene. Dystrophin is required for the organization of a complex consisting of dystroglycans, sarcoglycans, dystrobrevins and syntrophins, known as the dystrophin-associated proteins complex (DAPC). In addition to muscle degeneration, cognitive impairment has been reported in DMD patients. To characterize a suitable model for studying the embryonic cerebral functions of dystrophin, we analyzed the expression patterns of dystrophins/DAPC in undifferentiated and differentiated embryonic neural stem/progenitor cells (NSPC). We found that NSPC express mRNAs for dystrophins Dp427, Dp140, Dp71 and Dp40; β-dystroglycan; α- and β-dystrobrevin; α1-, β1-, β2- and γ2-syntrophin; and β-, γ-, δ- and ε-sarcoglycan. Some of these were differentially regulated during neuronal or astrocytic differentiation. Interestingly, the protein expression levels of Dp140, β-dystroglycan and α2-dystrobrevin were also differentially regulated. Additionally, we found that proliferating NSPC and differentiated neurons and astrocytes show immuno-positive staining for dystrophins and β-dystroglycan. Our results show that dystrophins and DAPC components are expressed and regulated during the neuronal or astrocytic differentiation of NSPC, suggesting that these proteins may have different roles in the brain development.

杜兴氏肌营养不良症（DMD）是由肌营养不良蛋白基因突变引起的遗传性疾病。肌营养不良蛋白是由肌营养不良蛋白聚糖，肌糖蛋白，肌营养不良蛋白和肌营养蛋白组成的复合物的组织所必需的，称为肌营养不良蛋白相关蛋白复合物（DAPC）。除肌肉变性外，DMD患者中也有认知障碍的报道。为了表征研究肌营养不良蛋白的胚胎脑功能的合适模型，我们分析了肌营养不良蛋白/ DAPC在未分化和分化的胚胎神经干/祖细胞（NSPC）中的表达模式。我们发现NSPC表达营养不良蛋白Dp427，Dp140，Dp71和Dp40的mRNA。β-dystroglycan；α-和β-dystrobrevin；α1-，β1-，β2-和γ2-突触核蛋白；以及β-，γ-，δ-和ε-糖聚糖。其中一些在神经元或星形细胞分化过程中受到差异调节。有趣的是，Dp140，β-dystroglycan和α2-dystrobrevin的蛋白质表达水平也受到差异调节。此外，我们发现增殖的NSPC以及分化的神经元和星形胶质细胞对营养不良蛋白和β-营养不良聚糖表现出免疫阳性染色。我们的结果表明，营养不良蛋白和DAPC组分在NSPC的神经元或星形细胞分化过程中表达和调控，表明这些蛋白在大脑发育中可能具有不同的作用。我们发现增殖的NSPC以及分化的神经元和星形胶质细胞对营养不良蛋白和β-营养不良聚糖表现出免疫阳性染色。我们的结果表明，营养不良蛋白和DAPC组分在NSPC的神经元或星形细胞分化过程中表达和调控，表明这些蛋白在大脑发育中可能具有不同的作用。我们发现增殖的NSPC以及分化的神经元和星形胶质细胞对营养不良蛋白和β-营养不良聚糖表现出免疫阳性染色。我们的结果表明，营养不良蛋白和DAPC组分在NSPC的神经元或星形细胞分化过程中表达和调控，表明这些蛋白在大脑发育中可能具有不同的作用。