Utrophin haploinsufficiency does not worsen the functional performance, resistance to eccentric contractions and force production of dystrophic mice. [PDF]
PLoS ONE, 2018 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
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iNOS is not responsible for RyR1 S-nitrosylation in mdx mice with truncated dystrophin
BMC Musculoskeletal Disorders, 2020 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
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L-type Ca2+ channel function is linked to dystrophin expression in mammalian muscle. [PDF]
PLoS ONE, 2008 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
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Diagnostics, 2023 This study aimed to evaluate cardiac function in a young mouse model of Duchenne muscular dystrophy (mdx) using cardiac magnetic resonance imaging (MRI) with feature tracking and self-gated magnetic resonance cine imaging.
Junpei Ueda, Shigeyoshi Saito
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Differential requirement for utrophin in the induced pluripotent stem cell correction of muscle versus fat in muscular dystrophy mice. [PDF]
PLoS ONE, 2011 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
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Skeletal muscle fibrosis in the mdx/utrn+/- mouse validates its suitability as a murine model of Duchenne muscular dystrophy. [PDF]
PLoS ONE, 2015 Various therapeutic approaches have been studied for the treatment of Duchenne muscular dystrophy (DMD), but none of these approaches have led to significant long-term effects in patients.
Kelly M Gutpell +2 more
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BMC Cardiovascular Disorders, 2022 Background Bone marrow mesenchymal stem cells (BMSCs) are commonly used in regenerative medicine. However, it is not clear whether transplantation of BMSCs can improve cardiac function of the X-Linked Muscular Dystrophy Mice (mdx) and how to detect it ...
Xiao Liu +6 more
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Sequencing protocols to genotype mdx, mdx4cv, and mdx5cv mice [PDF]
Muscle & Nerve, 2010 AbstractCurrently available polymerase chain reaction (PCR) genotyping methods for point mutations in the mouse dystrophin gene can lead to false positives and result in wasted time and money due to breeding or treating the wrong mice. Here we describe a simple and accurate method for sequencing the point mutations in mdx, mdx4cv, and mdx5cv mice. This
Glen B. Banks +2 more
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Evaluation of skeletal and cardiac muscle function after chronic administration of thymosin beta-4 in the dystrophin deficient mouse. [PDF]
PLoS ONE, 2010 Thymosin beta-4 (Tbeta4) is a ubiquitous protein with many properties relating to cell proliferation and differentiation that promotes wound healing and modulates inflammatory mediators.
Christopher F Spurney +8 more
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Progression of kyphosis in mdx mice [PDF]
Journal of Applied Physiology, 2004 Spinal deformity in the form of kyphosis or kyphoscoliosis occurs in most patients with Duchenne muscular dystrophy (DMD), a fatal X-linked disorder caused by an absence of the subsarcolemmal protein dystrophin. Mdx mice, which also lack dystrophin, show thoracolumbar kyphosis that progresses with age.
Laws, Nicola, Hoey, Andrew
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