title: In vitro mouse model in Duchenne muscular dystrophy diagnosis using 50-MHz ultrasound waves.
abstract: Duchenne muscular dystrophy (DMD) is caused by the absence of dystrophin, the protein that plays a key mechanical role in maintaining muscle membrane integrity. One of the major consequences of dystrophin deficiency is the degeneration of muscle fibres, with a progressive loss in muscle strength. The objective of this research was to find an ultrasonic parameter sensitive to DMD, which could give relevant information related to microstructure if compared to traditional investigations such as morphometrical analysis. This "in vitro" study focused on the Mdx mouse model and investigated the potential differences between wild-type and dystrophin-deficient mice diaphragms. Using a 50MHz ultrasonic sensor built in our group, we recorded an increase in ultrasonic wave attenuation in the dystrophin-deficient samples in comparison with normal muscles. A correlation between attenuation, mouse age and the percentage of non-muscular proportion in muscle was observed. As Mdx mouse is the best animal model for DMD and reproduces the degenerative pattern observed in human DMD muscles, this approach could be a powerful tool for in vitro DMD investigation and, more generally, for the characterisation of muscle properties.
authors.affiliation: Institut d'Electronique du Sud, IES, CNRS UMR 5214, University Montpellier II, Place Eugène Bataillon, Montpellier, France. laux@lain.univ-montp2.fr
authors.forename: D
authors.initials: D
authors.lastname: Laux
authors.forename: H
authors.initials: H
authors.lastname: Blasco
authors.forename: J Y
authors.initials: JY
authors.lastname: Ferrandis
authors.forename: G
authors.initials: G
authors.lastname: Hugon
authors.forename: G
authors.initials: G
authors.lastname: Despaux
authors.forename: A
authors.initials: A
authors.lastname: Leydier
authors.forename: D
authors.initials: D
authors.lastname: Mornet
mesh_terms: D000818:Animals; D004195:Disease Models, Animal; D007150:Immunohistochemistry; D051379:Mice; D008810:Mice, Inbred C57BL; D018101:Mice, Inbred mdx; D018482:Muscle, Skeletal / Q000000981:diagnostic imaging; D009137:Muscular Dystrophy, Animal / Q000000981:diagnostic imaging*; D020388:Muscular Dystrophy, Duchenne / Q000000981:diagnostic imaging*; D014463:Ultrasonography