LAMA2-related congenital muscular dystrophy (CMD; LAMA2-MD), also referred to as merosin deficient CMD (MDC1A), is a severe neonatal onset muscle disease caused by recessive mutations in the LAMA2 gene. LAMA2 encodes laminin α2, a subunit of the extracellular matrix (ECM) oligomer laminin 211. There are currently no treatments for MDC1A, and there is an incomplete understanding of disease pathogenesis. Zebrafish, due to their high degree of genetic conservation with humans, large clutch sizes, rapid development, and optical clarity, have emerged as an excellent model system for studying rare Mendelian diseases. They are particularly suitable as a model for muscular dystrophy because they contain at least one orthologue to all major human MD genes, have muscle that is similar to human muscle in structure and function, and manifest obvious and easily measured MD related phenotypes. In this review article, we present the existing zebrafish models of MDC1A, and discuss their contribution to the understanding of MDC1A pathomechanisms and therapy development.

LAMA2相关的先天性肌营养不良（CMD; LAMA2-MD），也称为铁蛋白缺陷型CMD（MDC1A），是由LAMA2基因的隐性突变引起的严重的新生儿发作性肌肉疾病。LAMA2编码层粘连蛋白α2，它是细胞外基质（ECM）寡聚层粘连蛋白211的一个亚基。目前尚无MDC1A的治疗方法，并且对疾病的发病机理还不完全了解。斑马鱼由于具有高度的人类遗传保守性，大的离合大小，快速的发育和光学清晰度，已经成为研究稀有孟德尔疾病的优秀模型系统。它们特别适合作为肌肉营养不良的模型，因为它们对所有主要人类MD基因都含有至少一个直向同源物，其肌肉在结构和功能上与人类的肌肉相似，并表现出明显且易于测量的MD相关表型。在这篇综述文章中，我们介绍了MDC1A的现有斑马鱼模型，并讨论了它们对MDC1A致病机理和疗法发展的理解。