Results 51 to 60 of about 1,087,549 (254)

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

Aquaporin 4 Expression in the mdx Mouse Diaphragm

open access: yesACTA HISTOCHEMICA ET CYTOCHEMICA, 2011
Expression of aquaporin (AQP) 4 in the surface membranes of skeletal myofibers is well established; however, its functional significance is still unknown. The alterations of AQP4 expressions in dystrophic muscles at RNA and protein levels have been reported in various dystrophic muscles such as dystrophinopathy, dysferlinopathy, and sarcoglycanopathy ...
Takahiro Jimi   +6 more
openaire   +4 more sources

Natural disease history of the D2-mdx mouse model for Duchenne muscular dystrophy

open access: yesThe FASEB Journal, 2019
The C57BL/10ScSn‐Dmdmdx/J (BL10‐mdx) mouse has been the most commonly used model for Duchenne muscular dystrophy (DMD) for decades. Their muscle dysfunction and pathology is, however, less severe than in patients with DMD, which complicates preclinical ...
M. van Putten   +6 more
semanticscholar   +1 more source

Molecular and genetic mapping of the mouse mdx locus

open access: yesGenomics, 1988
mdx is an X-linked muscular dystrophy mutant of the mouse and a putative homolog of the human X-linked muscular dystrophy locus--Duchenne muscular dystrophy (DMD). Utilizing a C57BL/10/Mus Spretus interspecific cross in which the mdx mutation was segregating, we have constructed a detailed genetic map around the mdx locus on the mouse X chromosome.
Stephen D.M. Brown   +6 more
openaire   +2 more sources

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

Lipidomic Analyses Reveal Specific Alterations of Phosphatidylcholine in Dystrophic Mdx Muscle

open access: yesFrontiers in Physiology, 2022
In Duchenne muscular dystrophy (DMD), lack of dystrophin increases the permeability of myofiber plasma membranes to ions and larger macromolecules, disrupting calcium signaling and leading to progressive muscle wasting. Although the biological origin and
William J. Valentine   +15 more
doaj   +1 more source

Human Galectin-1 Improves Sarcolemma Stability and Muscle Vascularization in the mdx Mouse Model of Duchenne Muscular Dystrophy

open access: yesMolecular Therapy: Methods & Clinical Development, 2019
Duchenne muscular dystrophy (DMD) is a devastating disease caused by mutations in the dystrophin gene that result in the complete absence of dystrophin protein.
Ryan D. Wuebbles   +6 more
doaj   +1 more source

A sandwich ELISA kit reveals marked elevation of titin N‐terminal fragment levels in the urine of mdx mice

open access: yesAnimal Models and Experimental Medicine, 2022
The mdx mouse is a model of Duchenne muscular dystrophy (DMD), a fatal progressive muscle wasting disease caused by dystrophin deficiency, and is used most widely in preclinical studies.
Taku Shirakawa   +10 more
doaj   +1 more source

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