Results 31 to 40 of about 9,256 (223)

T-Cell-Dependent Fibrosis in the mdx Dystrophic Mouse [PDF]

Laboratory Investigation, 2000
In Duchenne muscular dystrophy patients, the pathological hallmark of the disease, namely, the chronic accumulation of sclerotic scar tissue in the interstitial space of skeletal muscle is attributed to manifestation of secondary pathological processes.
Morrison, J, Lu, Q, Pastoret, C, Partridge, T, Bou-Gharios, G   +4 more
openaire   +2 more sources

Mitochondrial Transplantation Therapy Ameliorates Muscular Dystrophy in mdx Mouse Model. [PDF]

Biomolecules
Duchenne muscular dystrophy is caused by loss of the dystrophin protein. This pathology is accompanied by mitochondrial dysfunction contributing to muscle fiber instability. It is known that mitochondria-targeted in vivo therapy mitigates pathology and improves the quality of life of model animals.
Dubinin MV, Mikheeva IB, Stepanova AE, Igoshkina AD, Cherepanova AA, Semenova AA, Sharapov VA, Kireev II, Belosludtsev KN.   +8 more
europepmc   +4 more sources

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

JACC: 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, Michelle L. Asp, PhD, Huiliang Zhang, PhD, Wang Wang, MD, PhD, Joseph M. Metzger, 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]

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, Joseph M Vitale, Qingshi Zhao, Joel S Schneider, Corey Chang, Aneela Altaf, Jennifer Michaels, Mantu Bhaumik, Robert Grange, Diego Fraidenraich   +9 more
doaj   +1 more source

Lipidomic Analyses Reveal Specific Alterations of Phosphatidylcholine in Dystrophic Mdx Muscle

Frontiers 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, William J. Valentine, Sherif A. Mostafa, Sherif A. Mostafa, Suzumi M. Tokuoka, Fumie Hamano, Fumie Hamano, Natsuko F. Inagaki, Joel Z. Nordin, Joel Z. Nordin, Norio Motohashi, Yoshihiro Kita, Yoshitsugu Aoki, Takao Shimizu, Hideo Shindou, Hideo Shindou   +15 more
doaj   +1 more source

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

Animal 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, Ayumu Ikushima, Nobuhiro Maruyama, Yoshinori Nambu, Hiroyuki Awano, Kayo Osawa, Kei Nirasawa, Yoichi Negishi, Hisahide Nishio, Shoji Fukushima, Masafumi Matsuo   +10 more
doaj   +1 more source

Aquaporin 4 Expression in the mdx Mouse Diaphragm

ACTA 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 ...
Hara, Hajime, Wakayama, Yoshihiro, Kojima, Hiroko, Inoue, Masahiko, Jimi, Takahiro, Iijima, Shoji, Masaki, Hisatsugu   +6 more
openaire   +3 more sources

Marginal level dystrophin expression improves clinical outcome in a strain of dystrophin/utrophin double knockout mice. [PDF]

PLoS ONE, 2010
Inactivation of all utrophin isoforms in dystrophin-deficient mdx mice results in a strain of utrophin knockout mdx (uko/mdx) mice. Uko/mdx mice display severe clinical symptoms and die prematurely as in Duchenne muscular dystrophy (DMD) patients.
Dejia Li, Yongping Yue, Dongsheng Duan
doaj   +1 more source

The use of urinary and kidney SILAM proteomics to monitor kidney response to high dose morpholino oligonucleotides in the mdx mouse

Toxicology Reports, 2015
Phosphorodiamidate morpholino oligonucleotides (PMO) are used as a promising exon-skipping gene therapy for Duchenne muscular dystrophy (DMD). One potential complication of high dose PMO therapy is its transient accumulation in the kidneys. Therefore new
Aiping Zhang, Kitipong Uaesoontrachoon, Conner Shaughnessy, Jharna R. Das, Sree Rayavarapu, Kristy J. Brown, Patricio E. Ray, Kanneboyina Nagaraju, John N. van den Anker, Eric P. Hoffman, Yetrib Hathout   +10 more
doaj   +1 more source

A comparison of the bone and growth phenotype of mdx, mdx:Cmah−/− and mdx:Utrn+/− murine models with the C57BL/10 wild-type mouse

Disease Models & Mechanisms, 2020
The muscular dystrophy X-linked (mdx) mouse is commonly used as a mouse model of Duchenne muscular dystrophy (DMD). Its phenotype is, however, mild, and other mouse models have been explored.
Claire L. Wood, Karla J. Suchacki, Rob van ’t Hof, Will P. Cawthorn, Scott Dillon, Volker Straub, Sze Choong Wong, Syed F. Ahmed, Colin Farquharson, Annemieke Aartsma-Rus, James Dowling, Maaike van Putten   +11 more
doaj   +1 more source