Results 1 to 10 of about 18,188 (219)

Characterization of Alzheimer's disease‐like neuropathology in Duchenne's muscular dystrophy using the DBA/2J mdx mouse model [PDF]

FEBS Open Bio, 2022
Duchenne muscular dystrophy (DMD) is a progressive muscle wasting disorder caused by a mutation in the dystrophin gene. In addition to muscle pathology, some patients with DMD will exhibit cognitive impairments with severity being linked to age and type ...
Grant C. Hayward, Daniela Caceres, Emily N. Copeland, Bradley J. Baranowski, Ahmad Mohammad, Kennedy C. Whitley, Val A. Fajardo, Rebecca E. K. MacPherson   +7 more
doaj   +2 more sources

Intrinsic Muscle Stem Cell Dysfunction Contributes to Impaired Regeneration in the mdx Mouse [PDF]

Journal of Cachexia, Sarcopenia and Muscle
Background Duchenne muscular dystrophy (DMD) is a devastating disease characterized by progressive muscle wasting that leads to diminished lifespan. In addition to the inherent weakness of dystrophin‐deficient muscle, the dysfunction of resident muscle ...
Marie E. Esper, Caroline E. Brun, Alexander Y. T. Lin, Peter Feige, Marie J. Catenacci, Marie‐Claude Sincennes, Morten Ritso, Michael A. Rudnicki   +7 more
doaj   +2 more sources

Determination of qPCR reference genes suitable for normalizing gene expression in a novel model of Duchenne muscular dystrophy, the D2-mdx mouse. [PDF]

PLoS ONE
Duchenne muscular dystrophy (DMD) is a X-linked neuromuscular disorder arising from mutations in the dystrophin gene, leading to a progressive muscle wasting and disability.
Brigida Boccanegra, Roberta Lenti, Paola Mantuano, Elena Conte, Lisamaura Tulimiero, Richard J Piercy, Ornella Cappellari, John C W Hildyard, Annamaria De Luca   +8 more
doaj   +2 more sources

Chronic N‐acetyl cysteine treatment does not improve respiratory system performance in the mdx mouse model of Duchenne muscular dystrophy [PDF]

Experimental Physiology
Duchenne muscular dystrophy (DMD) is characterised by respiratory muscle injury, inflammation, fibrosis and weakness, ultimately culminating in respiratory failure.
Michael N. Maxwell, Anthony L. Marullo, Esther Valverde‐Pérez, Aoife D. Slyne, Ben T. Murphy, Ken D. O'Halloran   +5 more
doaj   +2 more sources

Aberrant location of inhibitory synaptic marker proteins in the hippocampus of dystrophin-deficient mice [PDF]

, 2014
Duchenne muscular dystrophy (DMD) is a neuromuscular disease that arises from mutations in the dystrophin-encoding gene. Apart from muscle pathology, cognitive impairment, primarily of developmental origin, is also a significant component of the disorder.
Gorecki, Dariusz C., Krasowska, Elzbieta, Swinny, Jerome D., Zablocki, Krzysztof   +3 more
core   +14 more sources

Sarco(endo)plasmic reticulum Ca2+-ATPase function is impaired in skeletal and cardiac muscles from young DBA/2J mdx mice

iScience, 2022
Summary: The DBA/2J (D2) mdx mouse is a more severe model of Duchenne muscular dystrophy when compared to the traditional C57BL/10 (C57) mdx mouse. Here, we questioned whether sarco(endo)plasmic reticulum Ca2+-ATPase (SERCA) function would differ in ...
Riley E.G. Cleverdon, Jessica L. Braun, Mia S. Geromella, Kennedy C. Whitley, Daniel M. Marko, Sophie I. Hamstra, Brian D. Roy, Rebecca E.K. MacPherson, Val A. Fajardo   +8 more
doaj   +1 more source

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, William T Hrinivich, Lisa M Hoffman   +2 more
doaj   +1 more source

Peptide-conjugated phosphodiamidate oligomer-mediated exon skipping has benefits for cardiac function in mdx and Cmah-/-mdx mouse models of Duchenne muscular dystrophy. [PDF]

PLoS ONE, 2018
Cardiac failure is a major cause of mortality in patients with Duchenne muscular dystrophy (DMD). Antisense-mediated exon skipping has the ability to correct out-of-frame mutations in DMD to produce truncated but functional dystrophin.
Alison M Blain, Elizabeth Greally, Graham McClorey, Raquel Manzano, Corinne A Betts, Caroline Godfrey, Liz O'Donovan, Thibault Coursindel, Mike J Gait, Matthew J Wood, Guy A MacGowan, Volker W Straub   +11 more
doaj   +1 more source

Quantitative T2 combined with texture analysis of nuclear magnetic resonance images identify different degrees of muscle involvement in three mouse models of muscle dystrophy: mdx, Largemyd and mdx/Largemyd.

PLoS ONE, 2015
Quantitative nuclear magnetic resonance imaging (MRI) has been considered a promising non-invasive tool for monitoring therapeutic essays in small size mouse models of muscular dystrophies.
Aurea B Martins-Bach, Jackeline Malheiros, Béatrice Matot, Poliana C M Martins, Camila F Almeida, Waldir Caldeira, Alberto F Ribeiro, Paulo Loureiro de Sousa, Noura Azzabou, Alberto Tannús, Pierre G Carlier, Mariz Vainzof   +11 more
doaj   +1 more source

Increased levels of interleukin-6 exacerbate the dystrophic phenotype in mdx mice [PDF]

, 2015
Duchenne muscular dystrophy (DMD) is characterized by progressive lethal muscle degeneration and chronic inflammatory response. The mdx mouse strain has served as the animal model for human DMD. However, while DMD patients undergo extensive necrosis, the
Berardinelli, Maria Grazia, Camilli, Carlotta, Catizone, Angela, De Benedetti, Fabrizio, Forcina, Laura, Musarò, Antonio, Nicoletti, Carmine, Pelosi, Laura, Rizzuto, Emanuele, Spelta, Elisa, Testa, Erika   +10 more
core   +2 more sources