Muscle stem cells (MuSCs), known as satellite cells (SCs) have an incredible ability to regenerate, which enables the maintenance and growth of muscle tissue. In response to damaging stimuli, SCs are activated, proliferate, differentiate, and fuse to repair or generate a new muscle fiber. However, dystrophic muscles are characterized by poor muscle regeneration along with chronic inflammation and fibrosis. Indications for SC involvement in muscular dystrophy pathologies are accumulating, but their contribution to muscle pathophysiology is not precisely understood. In congenital muscular dystrophy type 1A (LAMA2-CMD), mutations in Lama2 gene cause either complete or partial absence in laminin-211 protein. Laminin-211 functions as a link between muscle extracellular matrix (ECM) and two adhesion systems in the sarcolemma; one is the well-known dystrophin–glycoprotein complex (DGC), and the second is the integrin complex. Because of its protein interactions and location, laminin-211 has a crucial role in muscle function and survival by maintaining sarcolemma integrity. In addition, laminin-211 is expressed in SCs and suggested to have a role in SC proliferation and differentiation. Downstream to the primary defect in laminin-211, several secondary genes and pathways accelerate disease mechanism, while at the same time there are unsuccessful attempts to regenerate as compensation for the dystrophic process. Lately, next-generation sequencing platforms have advanced our knowledge about the secondary events occurring in various diseases, elucidate the pathophysiology, and characterize new essential targets for development of new treatment strategies. This review will mainly focus on SC contribution to impaired regeneration in muscular dystrophies and specifically new findings suggesting SC involvement in LAMA2-CMD pathology.

称为卫星细胞（SCs）的肌肉干细胞（MuSC）具有令人难以置信的再生能力，能够维持和生长肌肉组织。响应于破坏性刺激，SC被激活，增殖，分化并融合以修复或产生新的肌纤维。然而，营养不良性肌肉的特征在于不良的肌肉再生以及慢性炎症和纤维化。SC参与肌营养不良症病理学的指征正在积累，但尚不清楚它们对肌肉病理生理学的贡献。在1A型先天性肌营养不良症（LAMA2-CMD）中，喇嘛2基因导致层粘连蛋白211蛋白完全或部分缺失。层粘连蛋白211充当肌肉细胞外基质（ECM）和肌膜中两个黏附系统之间的链接；一种是众所周知的肌营养不良蛋白-糖蛋白复合物（DGC），另一种是整联蛋白复合物。由于层粘连蛋白211的蛋白质相互作用和位置，它通过保持肌膜完整性在肌肉功能和存活中起关键作用。另外，层粘连蛋白211在SC中表达，并提示其在SC的增殖和分化中起作用。在层粘连蛋白211的主要缺陷的下游，一些次级基因和途径加速了疾病的机理，但同时也没有成功进行再生以弥补营养不良过程的尝试。最近，下一代测序平台提高了我们对各种疾病中继发事件的认识，阐明了病理生理，并为开发新的治疗策略确定了新的基本靶标。这项审查将主要侧重于SC​​对肌肉营养不良的受损再生的贡献，特别是表明SC参与LAMA2-CMD病理的新发现。