| First Author | Holmberg J | Year | 2014 |
| Journal | Front Aging Neurosci | Volume | 6 |
| Pages | 155 | PubMed ID | 25071564 |
| Mgi Jnum | J:310586 | Mgi Id | MGI:6763554 |
| Doi | 10.3389/fnagi.2014.00155 | Citation | Holmberg J, et al. (2014) Laminin alpha2 Chain-Deficiency is Associated with microRNA Deregulation in Skeletal Muscle and Plasma. Front Aging Neurosci 6:155 |
| abstractText | microRNAs (miRNAs) are widespread regulators of gene expression, but little is known of their potential roles in congenital muscular dystrophy type 1A (MDC1A). MDC1A is a severe form of muscular dystrophy caused by mutations in the gene encoding laminin alpha2 chain. To gain insight into the pathophysiological roles of miRNAs associated with MDC1A pathology, laminin alpha2 chain-deficient mice were evaluated by quantitative PCR. We demonstrate that expression of muscle-specific miR-1, miR-133a, and miR-206 is deregulated in laminin alpha2 chain-deficient muscle. Furthermore, expression of miR-223 and miR-21, associated with immune cell infiltration and fibrosis, respectively, is altered. Finally, we show that plasma levels of muscle-specific miRNAs are markedly elevated in laminin alpha2 chain-deficient mice and partially normalized in response to proteasome inhibition therapy. Altogether, our data suggest important roles for miRNAs in MDC1A pathology and we propose plasma levels of muscle-specific miRNAs as promising biomarkers for the progression of MDC1A. |
