Abstract
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, dy2J/dy2J, dy3K/dy3K, dyW/dyW, 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.
Highlights
A Family of Laminin α2 Chain-Deficient Mouse MutantsThe research on laminin α2 chain-deficient congenital muscular dystrophy (LAMA2CMD) 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
The use of animals for scientific purposes goes back to ancient Greece where philosophers and scientists such as Alcmaeon of Croton, Aristotle, and Erasistratus dissected animals for anatomical studies
Deficiency of dystrophin and β-sarcoglycan, respectively, severely worsened the phenotype of dy3K/dy3K mice (Gawlik et al, 2014; Table 5). These results suggested non redundant roles of laminin α2 and the dystrophin-glycoprotein complex (DGC) and a key impact of laminin-DGC axis on muscle homeostasis
Summary
The research on laminin α2 chain-deficient congenital muscular dystrophy (LAMA2CMD) 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. They enable researchers to test laborious, necessary routines, and the most creative scientific approaches in order to design therapy for this devastating disorder.
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