Combined MRI and ³¹P-MRS investigations of the ACTA1(H40Y) mouse model of nemaline myopathy show impaired muscle function and altered energy metabolism.

Combined MRI and ³¹P-MRS investigations of the ACTA1(H40Y) mouse model of nemaline myopathy show impaired muscle function and altered energy metabolism.

Nemaline myopathy (NM) is the most common disease entity among non-dystrophic skeletal muscle congenital diseases. Mutations in the skeletal muscle α-actin gene (ACTA1) account for ∼25% of all NM cases and are the most frequent cause of severe forms of NM. So far, the mechanisms underlying muscle we...

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Bibliographic Details
Main Authors: Charlotte Gineste, Yann Le Fur, Christophe Vilmen, Arnaud Le Troter, Emilie Pecchi, Patrick J Cozzone, Edna C Hardeman, David Bendahan, Julien Gondin
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2013-01-01
Series:PLoS ONE
Online Access:http://europepmc.org/articles/PMC3629063?pdf=render

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http://europepmc.org/articles/PMC3629063?pdf=render

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