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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Detalles Bibliográficos
Autores principales: Gineste, Charlotte, Duhamel, Guillaume, Le Fur, Yann, Vilmen, Christophe, Cozzone, Patrick J., Nowak, Kristen J., Bendahan, David, Gondin, Julien
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Public Library of Science 2013
Materias:
Research Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3748127/
https://www.ncbi.nlm.nih.gov/pubmed/23977274
http://dx.doi.org/10.1371/journal.pone.0072294

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3748127/
https://www.ncbi.nlm.nih.gov/pubmed/23977274
http://dx.doi.org/10.1371/journal.pone.0072294

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