title: Histological parameters for the quantitative assessment of muscular dystrophy in the mdx-mouse.
abstract: Duchenne muscular dystrophy is a severe X-linked hereditary disease caused by the absence of functional dystrophin. The dystrophin-deficient mdx-mouse strain is a widely used animal model for dystrophin-deficiency. Several therapeutic approaches for muscular dystrophy have been proposed by different laboratories. In order to compare the efficacy of these therapies in the mdx-mouse, it is essential to implement standardized protocols for the assessment of functional and histological parameters in this mouse model. Here, we determine that the minimal 'Feret's diameter' is a geometrical parameter that allows for reliable measure of muscle fiber cross-sectional size. Using this geometrical parameter we calculate variance coefficients of the muscle fiber size and provide reference values for the quantitative assessment of dystrophic symptoms in frequently investigated muscles of wild-type and mdx-mouse. In addition, we compare the variance coefficients of the muscle fiber size with the percentage of muscle fibers with centralized nuclei; another histological hallmark of muscular dystrophy.
authors.affiliation: MyoContract Ltd, Hammerstrasse 25, CH-4410 Liestal, Switzerland. alexandre.briguet@myocontract.com
authors.forename: Alexandre
authors.initials: A
authors.lastname: Briguet
authors.forename: Isabelle
authors.initials: I
authors.lastname: Courdier-Fruh
authors.forename: Mark
authors.initials: M
authors.lastname: Foster
authors.forename: Thomas
authors.initials: T
authors.lastname: Meier
authors.forename: Josef P
authors.initials: JP
authors.lastname: Magyar
mesh_terms: D000367:Age Factors; D000818:Animals; D004195:Disease Models, Animal; D006652:Histological Techniques / Q000379:methods; D007211:Indoles; D051379:Mice; D008810:Mice, Inbred C57BL; D018101:Mice, Inbred mdx; D018485:Muscle Fibers, Skeletal / Q000378:metabolism / Q000473:pathology*; D018482:Muscle, Skeletal / Q000378:metabolism / Q000473:pathology*; D009137:Muscular Dystrophy, Animal / Q000473:pathology*; D012016:Reference Values
chemical_list: D007211:Indoles; C007293:DAPI