Exploring the respiratory efficacy of combined chronic glucocorticoid and antioxidant interventions in the mdx mouse: The PREDNAC trial [PDF]
Duchenne muscular dystrophy (DMD) is characterized by respiratory muscle injury and weakness, ultimately leading to respiratory failure. Impaired respiratory muscle performance, fibrosis and inflammation in early disease are evident in the dystrophin ...
Michael N. Maxwell +3 more
doaj +3 more sources
Immunoproteasome Inhibition Positively Impacts the Gut‐Muscle Axis in Duchenne Muscular Dystrophy [PDF]
Background Duchenne Muscular Dystrophy (DMD) features immune‐muscle crosstalk, where muscle fibre degeneration enhances pro‐inflammatory macrophage infiltration, worsening inflammation and impairing regeneration.
Andrea Farini +17 more
doaj +2 more sources
Dystrophic Skeletal Muscle Phenotypes Can Be Horizontally Transferred via Fecal Microbiome Transplantations. [PDF]
We sought to determine a causal role for the microbiota in promoting dystrophic muscle characteristics by performing intra/inter‐genotype fecal microbiota transplantations (FMT) between wildtype (C57BL/10) and mdx (C57BL/10ScSn‐Dmdmdx/J) mice. We found that transplantation of mdx microbiotas into a wildtype mouse induced an mdx‐like muscle phenotype ...
Butcher J +5 more
europepmc +2 more sources
Exploring Desmin as a Potential Modifier in Duchenne Muscular Dystrophy-Associated Cardiomyopathy. [PDF]
ABSTRACT Aim Duchenne muscular dystrophy (DMD), a rare X‐linked genetic disorder, is affecting skeletal and cardiac muscles due to the loss of the dystrophin protein. Modifier proteins, whose expression is altered in DMD patients, may influence disease progression.
Guennec BE +12 more
europepmc +2 more sources
Summary: The DBA/2J (D2) mdx mouse is a more severe model of Duchenne muscular dystrophy when compared to the traditional C57BL/10 (C57) mdx mouse. Here, we questioned whether sarco(endo)plasmic reticulum Ca2+-ATPase (SERCA) function would differ in ...
Riley E.G. Cleverdon +8 more
doaj +1 more source
Low-Level Laser Therapy (LLLT) in Dystrophin-Deficient Muscle Cells: Effects on Regeneration Capacity, Inflammation Response and Oxidative Stress. [PDF]
The present study evaluated low-level laser therapy (LLLT) effects on some physiological pathways that may lead to muscle damage or regeneration capacity in dystrophin-deficient muscle cells of mdx mice, the experimental model of Duchenne muscular ...
Aline Barbosa Macedo +7 more
doaj +1 more source
Utrophin haploinsufficiency does not worsen the functional performance, resistance to eccentric contractions and force production of dystrophic mice. [PDF]
The lack of dystrophin in Duchenne muscular dystrophy (DMD) compromises the integrity and function of muscle fibers. Skeletal muscles, except the diaphragm, do not undergo progressive degeneration in adult mdx mice due to compensatory mechanisms ...
Antoine Boulanger Piette +7 more
doaj +1 more source
iNOS is not responsible for RyR1 S-nitrosylation in mdx mice with truncated dystrophin
Background Previous research indicated that nitric oxide synthase (NOS) is the key molecule for S-nitrosylation of ryanodine receptor 1 (RyR1) in DMD model mice (mdx mice) and that both neuronal NOS (nNOS) and inducible NOS (iNOS) might contribute to the
Ken’ichiro Nogami +7 more
doaj +1 more source
L-type Ca2+ channel function is linked to dystrophin expression in mammalian muscle. [PDF]
BACKGROUND: In dystrophic mdx skeletal muscle, aberrant Ca2+ homeostasis and fibre degeneration are found. The absence of dystrophin in models of Duchenne muscular dystrophy (DMD) has been connected to altered ion channel properties e.g.
Oliver Friedrich +4 more
doaj +1 more source
Differential requirement for utrophin in the induced pluripotent stem cell correction of muscle versus fat in muscular dystrophy mice. [PDF]
Duchenne muscular dystrophy (DMD) is an incurable degenerative muscle disorder. We injected WT mouse induced pluripotent stem cells (iPSCs) into mdx and mdx∶utrophin mutant blastocysts, which are predisposed to develop DMD with an increasing degree of ...
Amanda J Beck +9 more
doaj +1 more source

