Results 51 to 60 of about 1,104,554 (244)

Dystrophin Stabilizes α3- But Not α7-Containing Nicotinic Acetylcholine Receptor Subtypes at the Postsynaptic Apparatus in the Mouse Superior Cervical Ganglion

open access: yesNeurobiology of Disease, 2002
The nicotinic acetylcholine receptor (nAChR) subtypes were characterized in the superior cervical ganglion (SCG) of wild-type and dystrophin-lacking mdx mice.
Arianna Del Signore   +4 more
doaj   +1 more source

Increased circulating levels of interleukin-6 induce perturbation in redox-regulated signaling cascades in muscle of dystrophic mice [PDF]

open access: yes, 2017
Duchenne muscular dystrophy (DMD) is an X-linked genetic disease in which dystrophin gene is mutated, resulting in dysfunctional or absent dystrophin protein.
Forcina, Laura   +4 more
core   +3 more sources

Data on uncoupling protein-3 levels, hypoxia, low flow ischemia, and insulin stimulation in dystrophin-deficient mdx mouse hearts

open access: yesData in Brief, 2018
The data contain body weights, plasma free fatty acids concentrations and cardiac uncoupling protein-3 protein levels for wild-type and mdx mice. The data provide heart rates, left ventricular contractile functions, coronary flow, phosphocreatine ...
Wen Zhang   +4 more
doaj   +1 more source

Improving translational studies: lessons from rare neuromuscular diseases [PDF]

open access: yes, 2015
Animal models play a key role in the development of novel treatments for human disease. This is particularly true for rare diseases – defined as disorders that affect less than 1 in 2000 people in the human population – for which, very often, there are ...
Wells, D J
core   +3 more sources

Klotho gene silencing promotes pathology in the mdx mouse model of Duchenne muscular dystrophy. [PDF]

open access: bronzeHuman Molecular Genetics, 2016
Michelle Wehling-Henricks   +8 more
semanticscholar   +2 more sources

Notch signaling deficiency underlies age-dependent depletion of satellite cells in muscular dystrophy

open access: yesDisease Models & Mechanisms, 2014
Duchenne muscular dystrophy (DMD) is a devastating disease characterized by muscle wasting, loss of mobility and death in early adulthood. Satellite cells are muscle-resident stem cells responsible for the repair and regeneration of damaged muscles.
Chunhui Jiang   +5 more
doaj   +1 more source

Revertant fibres and dystrophin traces in Duchenne muscular dystrophy: Implication for clinical trials [PDF]

open access: yes, 2010
Duchenne muscular dystrophy (DMD) is characterised by the absence of dystrophin in muscle biopsies, although residual dystrophin can be present, either as dystrophin-positive (revertant) fibres or traces.
Arechavala-Gomeza, V   +12 more
core   +1 more source

Microtubule-Mediated Misregulation of Junctophilin-2 Underlies T-Tubule Disruptions and Calcium Mishandling in mdx Mice

open access: yesJACC: Basic to Translational Science, 2016
Cardiac myocytes from the mdx mouse, the mouse model of Duchenne muscular dystrophy, exhibit t-tubule disarray and increased calcium sparks, but a unifying molecular mechanism has not been elucidated.
Kurt W. Prins, MD, PhD   +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]

open access: yesPLoS 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
doaj   +1 more source

Cytokines and chemokines as regulators of skeletal muscle inflammation: presenting the case of Duchenne muscular dystrophy [PDF]

open access: yes, 2013
Duchenne muscular dystrophy is a severe inherited muscle disease that affects 1 in 3500 boys worldwide. Infiltration of skeletal muscle by inflammatory cells is an important facet of disease pathophysiology and is strongly associated with disease ...
De Bleecker, Jan, De Paepe, Boel
core   +3 more sources

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