Laminin-111 Protein Therapy Reduces Muscle Pathology and Improves Viability of a Mouse Model of Merosin-Deficient Congenital Muscular Dystrophy

Merosin-deficient congenital muscular dystrophy type 1A (MDC1A) is a lethal muscle-wasting disease that is caused by mutations in the LAMA2 gene, resulting in the loss of laminin-α2 protein. MDC1A patients exhibit severe muscle weakness from birth, are confined to a wheelchair, require ventilator as...

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Bibliographische Detailangaben
Hauptverfasser: Rooney, Jachinta E., Knapp, Jolie R., Hodges, Bradley L., Wuebbles, Ryan D., Burkin, Dean J.
Format: Artigo
Sprache:Inglês
Veröffentlicht: American Society for Investigative Pathology 2012
Schlagworte:
Regular Article
Online Zugang:https://ncbi.nlm.nih.gov/pmc/articles/PMC3349899/
https://ncbi.nlm.nih.gov/pubmed/22322301
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.ajpath.2011.12.019
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