In addition to muscle disease, defects in processing and assembly of the dystrophin-glycoprotein complex (DGC) are associated with a spectrum of brain abnormalities ranging from mild cognitive impairment (MCI) to neuronal migration disorders. In brain, the DGC is involved in the organisation of GABA(A) receptors (GABA(A)Rs) and aquaporin-4 (AQP4)-containing protein complexes in neurons and glia, respectively. During development, defects in the glycosylation of α-dystroglycan that impair its ability to interact with the extracellular matrix (ECM) are frequently associated with cobblestone lissencephaly and mental retardation. Furthermore, mutations in the gene encoding ɛ-sarcoglycan (SGCE) cause the neurogenic movement disorder myoclonus dystonia syndrome. In this review, we describe recent progress in defining distinct roles for the DGC in neurons and glia.

中文翻译：

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大脑发育和疾病中的肌营养不良蛋白-糖蛋白复合物

除了肌肉疾病，抗肌萎缩蛋白-糖蛋白复合物 (DGC) 的加工和组装缺陷与从轻度认知障碍 (MCI) 到神经元迁移障碍的一系列大脑异常有关。在大脑中，DGC 分别参与神经元和胶质细胞中 GABA(A) 受体 (GABA(A)Rs) 和水通道蛋白 4 (AQP4) 蛋白复合物的组织。在发育过程中，α-肌营养不良聚糖的糖基化缺陷会损害其与细胞外基质 (ECM) 相互作用的能力，这通常与鹅卵石无脑畸形和智力低下有关。此外，编码 ɛ-肌聚糖 (SGCE) 的基因突变导致神经源性运动障碍肌阵挛肌张力障碍综合征。在本次审查中，