Results 1 to 10 of about 1,087,549 (254)

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   +3 more sources

Detailed genetic and functional analysis of the hDMDdel52/mdx mouse model. [PDF]

PLoS One, 2020
Duchenne muscular dystrophy (DMD) is a severe, progressive neuromuscular disorder caused by reading frame disrupting mutations in the DMD gene leading to absence of functional dystrophin.
Yavas A, Weij R, van Putten M, Kourkouta E, Beekman C, Puoliväli J, Bragge T, Ahtoniemi T, Knijnenburg J, Hoogenboom ME, Ariyurek Y, Aartsma-Rus A, van Deutekom J, Datson N.   +13 more
europepmc   +8 more sources

Characterisation of Progressive Skeletal Muscle Fibrosis in the Mdx Mouse Model of Duchenne Muscular Dystrophy: An In Vivo and In Vitro Study. [PDF]

Int J Mol Sci, 2022
Duchenne muscular dystrophy (DMD) is a rare genetic disease leading to progressive muscle wasting, respiratory failure, and cardiomyopathy. Although muscle fibrosis represents a DMD hallmark, the organisation of the extracellular matrix and the molecular
Giovarelli M, Arnaboldi F, Zecchini S, Cornaghi LB, Nava A, Sommariva M, Clementi EGI, Gagliano N.   +7 more
europepmc   +2 more sources

Partial Restoration of Brain Dystrophin and Behavioral Deficits by Exon Skipping in the Muscular Dystrophy X-Linked (mdx) Mouse. [PDF]

Ann Neurol, 2022
Duchenne muscular dystrophy is associated with various degrees of cognitive impairment and behavioral disturbances. Emotional and memory deficits also constitute reliable outcome measures to assess efficacy of treatments in the mdx mouse lacking the ...
Zarrouki F, Relizani K, Bizot F, Tensorer T, Garcia L, Vaillend C, Goyenvalle A.   +6 more
europepmc   +2 more sources

Lipocalin 2 Influences Bone and Muscle Phenotype in the MDX Mouse Model of Duchenne Muscular Dystrophy. [PDF]

Int J Mol Sci, 2022
Lipocalin 2 (Lcn2) is an adipokine involved in bone and energy metabolism. Its serum levels correlate with bone mechanical unloading and inflammation, two conditions representing hallmarks of Duchenne Muscular Dystrophy (DMD).
Ponzetti M, Ucci A, Maurizi A, Giacchi L, Teti A, Rucci N.   +5 more
europepmc   +2 more sources

Exploring the Therapeutic Potential of Ectoine in Duchenne Muscular Dystrophy: Comparison with Taurine, a Supplement with Known Beneficial Effects in the mdx Mouse. [PDF]

Int J Mol Sci, 2022
Duchenne Muscular Dystrophy (DMD) is a debilitating muscle disorder that condemns patients to year-long dependency on glucocorticoids. Chronic glucocorticoid use elicits many unfavourable side-effects without offering satisfying clinical improvement ...
Merckx C, Zschüntzsch J, Meyer S, Raedt R, Verschuere H, Schmidt J, De Paepe B, De Bleecker JL.   +7 more
europepmc   +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.
Dubinin MV, Mikheeva IB, Stepanova AE, Igoshkina AD, Cherepanova AA, Semenova AA, Sharapov VA, Kireev II, Belosludtsev KN.   +8 more
europepmc   +2 more sources

The D2.mdx mouse as a preclinical model of the skeletal muscle pathology associated with Duchenne muscular dystrophy. [PDF]

Sci Rep, 2020
Duchenne muscular dystrophy (DMD) is an X-linked, lethal muscle degenerative disease caused by loss of dystrophin protein. DMD has no cure and few treatment options. Preclinical efforts to identify potential DMD therapeutics have been hampered by lack of
Hammers DW, Hart CC, Matheny MK, Wright LA, Armellini M, Barton ER, Sweeney HL.   +6 more
europepmc   +2 more sources

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   +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