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MicroRNAs (miRNAs) are short non-coding RNAs that modulate gene expression post-transcriptionally. Current evidence suggests that miR-21 plays a significant role in the progression of fibrosis in muscle diseases. Laminin-deficient congenital muscular dystrophy (LAMA2-CMD) is a severe form of congenital muscular dystrophy caused by mutations in the gene encoding laminin α2 chain. Mouse models  dy   3K   /dy   3K   and  dy   2J   /dy   2J  , respectively, adequately mirror severe and milder forms of LAMA2-CMD. Both human and mouse LAMA2-CMD muscles are characterized by extensive fibrosis and considering that fibrosis is the final step that destroys muscle during the disease course, anti-fibrotic therapies may be effective strategies for prevention of LAMA2-CMD. We have previously demonstrated a significant up-regulation of the pro-fibrotic miR-21 in  dy   3K   /dy   3K   and  dy   2J   /dy   2J   skeletal muscle. Hence, the objective of this study was to explore if absence of miR-21 reduces fibrogenesis and improves the phenotype of LAMA2-CMD mice. Thus, we generated  dy   3K   /dy   3K   and  dy   2J   /dy   2J   mice devoid of miR-21 ( dy   3K   / miR-21 and  dy   2J   / miR-21 mice, respectively). However, the muscular dystrophy phenotype of  dy   3K  /miR-21 and  dy   2J  /miR-21 double knock-out mice was not improved compared to  dy   3K   /dy   3K   or  dy   2J   /dy   2J   mice, respectively. Mice displayed the same body weight, dystrophic muscles (with fibrosis) and impaired muscle function. These data indicate that miR-21 may not be involved in the development of fibrosis in LAMA2-CMD.

Absence of microRNA-21 does not reduce muscular dystrophy in mouse models of LAMA2-CMD