Impaired fetal muscle development and JAK-STAT activation mark disease onset and progression in a mouse model for merosin-deficient congenital muscular dystrophy

Merosin-deficient congenital muscular dystrophy type 1A (MDC1A) is a dramatic neuromuscular disease in which crippling muscle weakness is evident from birth. Here, we use the dy(W) mouse model for human MDC1A to trace the onset of the disease during development in utero. We find that myotomal and pr...

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Detalhes bibliográficos
Publicado no:Hum Mol Genet
Main Authors: Nunes, Andreia M., Wuebbles, Ryan D., Sarathy, Apurva, Fontelonga, Tatiana M., Deries, Marianne, Burkin, Dean J., Thorsteinsdóttir, Sólveig
Formato: Artigo
Idioma:Inglês
Publicado em: Oxford University Press 2017
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Articles
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC6075618/
https://ncbi.nlm.nih.gov/pubmed/28334989
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1093/hmg/ddx083
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