| First Author | Van Ry PM | Year | 2014 |
| Journal | Hum Mol Genet | Volume | 23 |
| Issue | 2 | Pages | 383-96 |
| PubMed ID | 24009313 | Mgi Jnum | J:204520 |
| Mgi Id | MGI:5532763 | Doi | 10.1093/hmg/ddt428 |
| Citation | Van Ry PM, et al. (2014) Laminin-111 improves muscle repair in a mouse model of merosin-deficient congenital muscular dystrophy. Hum Mol Genet 23(2):383-96 |
| abstractText | Merosin-deficient congenital muscular dystrophy type 1A (MDC1A) is a severe and fatal muscle-wasting disease with no cure. MDC1A patients and the dy(W-/-) mouse model exhibit severe muscle weakness, demyelinating neuropathy, failed muscle regeneration and premature death. We have recently shown that laminin-111, a form of laminin found in embryonic skeletal muscle, can substitute for the loss of laminin-211/221 and prevent muscle disease progression in the dy(W-/-) mouse model. What is unclear from these studies is whether laminin-111 can restore failed regeneration to laminin-alpha2-deficient muscle. To investigate the potential of laminin-111 protein therapy to improve muscle regeneration, laminin-111 or phosphate-buffered saline-treated laminin-alpha2-deficient muscle was damaged with cardiotoxin and muscle regeneration quantified. Our results show laminin-111 treatment promoted an increase in myofiber size and number, and an increased expression of alpha7beta1 integrin, Pax7, myogenin and embryonic myosin heavy chain, indicating a restoration of the muscle regenerative program. Together, our results show laminin-111 restores muscle regeneration to laminin-alpha2-deficient muscle and further supports laminin-111 protein as a therapy for the treatment of MDC1A. |
