Multimodal MRI and (31)P-MRS Investigations of the ACTA1(Asp286Gly) Mouse Model of Nemaline Myopathy Provide Evidence of Impaired In Vivo Muscle Function, Altered Muscle Structure and Disturbed Energy Metabolism

Nemaline myopathy (NM), the most common non-dystrophic congenital disease of skeletal muscle, can be caused by mutations in the skeletal muscle α-actin gene (ACTA1) (~25% of all NM cases and up to 50% of severe forms of NM). Muscle function of the recently generated transgenic mouse model carrying t...

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Detalhes bibliográficos
Main Authors: Gineste, Charlotte, Duhamel, Guillaume, Le Fur, Yann, Vilmen, Christophe, Cozzone, Patrick J., Nowak, Kristen J., Bendahan, David, Gondin, Julien
Formato: Artigo
Idioma:Inglês
Publicado em: Public Library of Science 2013
Assuntos:
Research Article
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC3748127/
https://ncbi.nlm.nih.gov/pubmed/23977274
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1371/journal.pone.0072294
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