AMPK Activation Stimulates Autophagy and Ameliorates Muscular Dystrophy in the mdx Mouse Diaphragm [PDF]
, 2012 Marion Pauly +13 more
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Comparative lipidomic and metabolomic profiling of mdx and severe mdx-apolipoprotein e-null mice
Skeletal MuscleDespite its notoriously mild phenotype, the dystrophin-deficient mdx mouse is the most common model of Duchenne muscular dystrophy (DMD). By mimicking a human DMD-associated metabolic comorbidity, hyperlipidemia, in mdx mice by inactivating the ...
Ram B. Khattri +7 more
doaj +1 more source
QuantiMus: A Machine Learning-Based Approach for High Precision Analysis of Skeletal Muscle Morphology. [PDF]
, 2019 Skeletal muscle injury provokes a regenerative response, characterized by the de novo generation of myofibers that are distinguished by central nucleation and re-expression of developmentally restricted genes.
Ayer, Rachel E +10 more
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Accumulation of Dystrophin-Positive Muscle Fibers and Improvement of Neuromuscular Junctions in mdx Mouse Muscles after Bone Marrow Transplantation under Different Conditions. [PDF]
Int J Mol Sci, 2023 Sokolova AV, Domnina AP, Mikhailov VM.
europepmc +1 more source
Identification of Disease Specific Pathways Using in Vivo SILAC Proteomics in Dystrophin Deficient mdx Mouse [PDF]
, 2013 Sree Rayavarapu +11 more
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PLoS ONEDuchenne muscular dystrophy (DMD) is an X-linked devastating disease caused by a lack of dystrophin which results in progressive muscle weakness. As muscle weakness progresses, respiratory insufficiency and hypoventilation result in significant morbidity
Marán Y Hernández Rodríguez +10 more
doaj +1 more source
Endocrine and Metabolic ScienceChronic inflammation is a feature of Duchenne muscular dystrophy (DMD), a degenerative striated muscle disease. Mineralocorticoid receptor (MR) antagonists (MRAs) have demonstrated clinical benefit on later onset DMD cardiomyopathy, and preclinical ...
Jeovanna Lowe +5 more
doaj +1 more source
Sunitinib inhibits STAT3 phosphorylation in cardiac muscle and prevents cardiomyopathy in the mdx mouse model of Duchenne muscular dystrophy. [PDF]
Hum Mol Genet, 2022 Oliveira-Santos A, Dagda M, Burkin DJ.
europepmc +1 more source
In vivo RNA editing of point mutations via RNA-guided adenosine deaminases. [PDF]
, 2019 We present in vivo sequence-specific RNA base editing via adenosine deaminases acting on RNA (ADAR) enzymes with associated ADAR guide RNAs (adRNAs). To achieve this, we systematically engineered adRNAs to harness ADARs, and comprehensively evaluated the
Chen, Genghao +7 more
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