scholarly journals Defects in mitochondrial localization and ATP synthesis in the mdx mouse model of Duchenne muscular dystrophy are not alleviated by PDE5 inhibition

2012 ◽  
Vol 22 (1) ◽  
pp. 153-167 ◽  
Author(s):  
J. M. Percival ◽  
M. P. Siegel ◽  
G. Knowels ◽  
D. J. Marcinek
Keyword(s):  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Mouse Model ◽  
Atp Synthesis ◽  
Mdx Mouse ◽  
Mitochondrial Localization ◽  
Pde5 Inhibition

scholarly journals Functional and Molecular Effects of Arginine Butyrate and Prednisone on Muscle and Heart in the mdx Mouse Model of Duchenne Muscular Dystrophy

PLoS ONE ◽  
2010 ◽  
Vol 5 (6) ◽  
pp. e11220 ◽  
Author(s):  
Alfredo D. Guerron ◽  
Rashmi Rawat ◽  
Arpana Sali ◽  
Christopher F. Spurney ◽  
Emidio Pistilli ◽  
...  
Keyword(s):  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Mouse Model ◽  
Mdx Mouse ◽  
Molecular Effects ◽  
Arginine Butyrate

scholarly journals Metabolomic Analyses Reveal Extensive Progenitor Cell Deficiencies in a Mouse Model of Duchenne Muscular Dystrophy

Metabolites ◽  
2018 ◽  
Vol 8 (4) ◽  
pp. 61 ◽  
Author(s):  
Josiane Joseph ◽  
Dong Cho ◽  
Jason Doles
Keyword(s):  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Mouse Model ◽  
Progenitor Cell ◽  
Treatment Options ◽  
Cell Types ◽  
Mdx Mouse ◽  
Mdx Mice ◽  
Metabolic Alterations ◽  
Potential Contributor

Duchenne muscular dystrophy (DMD) is a musculoskeletal disorder that causes severe morbidity and reduced lifespan. Individuals with DMD have an X-linked mutation that impairs their ability to produce functional dystrophin protein in muscle. No cure exists for this disease and the few therapies that are available do not dramatically delay disease progression. Thus, there is a need to better understand the mechanisms underlying DMD which may ultimately lead to improved treatment options. The muscular dystrophy (MDX) mouse model is frequently used to explore DMD disease traits. Though some studies of metabolism in dystrophic mice exist, few have characterized metabolic profiles of supporting cells in the diseased environment. Using nontargeted metabolomics we characterized metabolic alterations in muscle satellite cells (SCs) and serum of MDX mice. Additionally, live-cell imaging revealed MDX-derived adipose progenitor cell (APC) defects. Finally, metabolomic studies revealed a striking elevation of acylcarnitines in MDX APCs, which we show can inhibit APC proliferation. Together, these studies highlight widespread metabolic alterations in multiple progenitor cell types and serum from MDX mice and implicate dystrophy-associated metabolite imbalances in APCs as a potential contributor to adipose tissue disequilibrium in DMD.

Mss51 deletion increases endurance and ameliorates histopathology in the mdx mouse model of Duchenne muscular dystrophy

2021 ◽  
Vol 35 (2) ◽  
Author(s):  
Yazmin I. Rovira Gonzalez ◽  
Adam L. Moyer ◽  
Nicolas J. LeTexier ◽  
August D. Bratti ◽  
Siyuan Feng ◽  
...  
Keyword(s):  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Mouse Model ◽  
Mdx Mouse

scholarly journals Investigation of the effect of taurine supplementation on muscle taurine content in the mdx mouse model of Duchenne muscular dystrophy using chemically specific synchrotron imaging

The Analyst ◽  
2020 ◽  
Vol 145 (22) ◽  
pp. 7242-7251
Author(s):  
Jessica R. Terrill ◽  
Samuel M. Webb ◽  
Peter G. Arthur ◽  
Mark J. Hackett
Keyword(s):  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Mouse Model ◽  
Muscle Tissue ◽  
Mdx Mouse ◽  
Taurine Supplementation ◽  
Mouse Muscle ◽  
Synchrotron Imaging

Sulfur K-edge XANES was used to quantify changes in the taurine content of mouse muscle tissue in a model of muscular dystrophy. The changes could be associated with markers of disease pathology that were revealed by classical H&E histology.

scholarly journals Preclinical Studies in the mdx Mouse Model of Duchenne Muscular Dystrophy with the Histone Deacetylase Inhibitor Givinostat

2013 ◽  
Vol 19 (1) ◽  
pp. 79-87 ◽  
Author(s):  
Silvia Consalvi ◽  
Chiara Mozzetta ◽  
Paolo Bettica ◽  
Massimiliano Germani ◽  
Francesco Fiorentini ◽  
...  
Keyword(s):  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Mouse Model ◽  
Histone Deacetylase ◽  
Histone Deacetylase Inhibitor ◽  
Mdx Mouse ◽  
Preclinical Studies ◽  
Deacetylase Inhibitor
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