The research on laminin α2 chain-deficient congenital muscular dystrophy (LAMA2-CMD) advanced rapidly in the last few decades, largely due to availability of good mouse models for the disease and a strong interest in preclinical studies from scientists all over the world. These mouse models continue to provide a solid platform for understanding the LAMA2-CMD pathology. In addition, they enable researchers to test laborious, necessary routines, but also the most creative scientific approaches in order to design therapy for this devastating disorder. In this review we present animals belonging to the laminin α2 chain-deficient "dy/dy" mouse family (dy/dy, dy 2J/dy 2J, dy 3K/dy 3K, dy W/dy W, et al.) and a summary of the scientific progress they facilitated. We also raise a few questions that need to be addressed in order to maximize the usefulness of laminin α2 murine mutants and to further advance the LAMA2-CMD studies. We believe that research opportunities offered by the mouse models for LAMA2-CMD will continuously support our efforts to find a treatment for the disease.

中文翻译：

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层粘连蛋白α2链缺陷型小鼠突变体家族：促进LAMA2-CMD的研究。

在过去的几十年中，层粘连蛋白α2链缺陷型先天性肌营养不良症（LAMA2-CMD）的研究进展迅速，这主要是由于该疾病的良好小鼠模型的可获得性以及全世界科学家对临床前研究的浓厚兴趣。这些鼠标模型继续为理解LAMA2-CMD病理学提供了坚实的平台。此外，它们使研究人员能够测试费力的，必要的程序，而且还可以测试最具创新性的科学方法，以便为这种毁灭性疾病设计治疗方法。在这篇综述中，我们介绍属于层粘连蛋白α2链缺陷型“ dy / dy”小鼠家族的动物（dy / dy，dy 2J / dy 2J，dy 3K / dy 3K，dy W / dy W等）。他们促进的科学进步的摘要。为了使层粘连蛋白α2鼠突变体的效用最大化并进一步推进LAMA2-CMD研究，我们还提出了一些需要解决的问题。我们相信，小鼠模型为LAMA2-CMD提供的研究机会将继续支持我们为该疾病寻找治疗方法的努力。