Skip to Main content Skip to Navigation
Journal articles

Structural and Functional Alterations of Skeletal Muscle Microvasculature in Dystrophin-Deficient mdx Mice.

Abstract : Duchenne muscular dystrophy (DMD) is a progressive neuromuscular disease, caused by an absence of dystrophin, inevitably leading to death. Although muscle lesions are well characterized, blood vessel alterations that may have a major impact on muscle regeneration remain poorly understood. Our aim was to elucidate alterations of the vascular network organization, taking advantage of Flk1(GFP/+) crossed with mdx mice (model for human DMD where all blood vessels express green fluorescent protein) and functional repercussions using in vivo nuclear magnetic resonance, combining arterial spin-labeling imaging of perfusion, and (31)P-spectroscopy of phosphocreatine kinetics. For the first time, our study focused on old (12-month-old) mdx mice, displaying marked chronic muscle lesions, similar to the lesions observed in human DMD, in comparison to young-adult (3-month-old) mdx mice displaying only mild muscle lesions with no fibrosis. By using an original approach combining a specific animal model, state-of-the-art histology/morphometry techniques, and functional nuclear magnetic resonance, we demonstrated that the microvascular system is almost normal in young-adult in contrast to old mdx mice, displaying marked microvessel alterations, and the functional repercussions on muscle perfusion and bioenergetics after a hypoxic stress vary depending on stage of pathology. This original approach clarifies disease evolution and paves the way for setting up new diagnostic markers or therapeutic strategies.
Complete list of metadatas

Cited literature [30 references]  Display  Hide  Download

https://hal-pasteur.archives-ouvertes.fr/pasteur-01151848
Contributor : Claire Latroche <>
Submitted on : Wednesday, May 13, 2015 - 4:45:40 PM
Last modification on : Wednesday, August 19, 2020 - 11:18:08 AM
Long-term archiving on: : Wednesday, April 19, 2017 - 11:55:23 PM

File

Latroche_Am J Pathol_2015.pdf
Files produced by the author(s)

Licence


Distributed under a Creative Commons Attribution - NonCommercial 4.0 International License

Identifiers

Citation

Claire Latroche, Béatrice Matot, Aurea Martins-Bach, David Briand, Bénédicte Chazaud, et al.. Structural and Functional Alterations of Skeletal Muscle Microvasculature in Dystrophin-Deficient mdx Mice.. American Journal of Pathology, American Society for Investigative Pathology, 2015, 185 (9), pp.2482-94. ⟨10.1016/j.ajpath.2015.05.009⟩. ⟨pasteur-01151848⟩

Share

Metrics