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Immortalized myogenic cells from congenital muscular dystrophy type1A patients recapitulate aberrant caspase activation in pathogenesis: a new tool for MDC1A research

BACKGROUND: Congenital muscular dystrophy Type 1A (MDC1A) is a severe, recessive disease of childhood onset that is caused by mutations in the LAMA2 gene encoding laminin-α2. Studies with both mouse models and primary cultures of human MDC1A myogenic cells suggest that aberrant activation of cell de...

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Detalles Bibliográficos
Autores principales: Yoon, Soonsang, Stadler, Guido, Beermann, Mary Lou, Schmidt, Eric V, Windelborn, James A, Schneiderat, Peter, Wright, Woodring E, Miller, Jeffrey Boone
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2013
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3906772/
https://www.ncbi.nlm.nih.gov/pubmed/24314268
http://dx.doi.org/10.1186/2044-5040-3-28