Journal
HUMAN MOLECULAR GENETICS
Volume 28, Issue 16, Pages 2686-2695Publisher
OXFORD UNIV PRESS
DOI: 10.1093/hmg/ddz086
Keywords
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Funding
- Muscular Dystrophy Association (MDA) [23898]
- Parent Project Muscular Dystrophy (PPMD)
- National Institutes of Health (NIH)/National Institute of Arthritis and Musculoskeletal and Skin Diseases(NIAMS) [R01AR064338-01A1]
- MDA [240684]
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Duchenne muscular dystrophy (DMD) is a devastating X-linked disease affecting similar to 1 in 5000 males. DMD patients exhibit progressive muscle degeneration and weakness, leading to loss of ambulation and premature death from cardiopulmonary failure. We previously reported that mouse Laminin-111 (msLam-111) protein could reduce muscle pathology and improve muscle function in the mdx mouse model for DMD. In this study, we examined the ability of msLam-111 to prevent muscle disease progression in the golden retriever muscular dystrophy (GRMD) dog model of DMD. The msLam-111 protein was injected into the cranial tibial muscle compartment of GRMD dogs and muscle strength and pathology were assessed. The results showed that msLam-111 treatment increased muscle fiber regeneration and repair with improved muscle strength and reduced muscle fibrosis in the GRMD model. Together, these findings support the idea that Laminin-111 could serve as a novel protein therapy for the treatment of DMD.
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