Results 11 to 20 of about 38,496 (259)

Immunoproteasome Inhibition Positively Impacts the Gut‐Muscle Axis in Duchenne Muscular Dystrophy [PDF]

Journal of Cachexia, Sarcopenia and Muscle
Background Duchenne Muscular Dystrophy (DMD) features immune‐muscle crosstalk, where muscle fibre degeneration enhances pro‐inflammatory macrophage infiltration, worsening inflammation and impairing regeneration.
Andrea Farini, Francesco Strati, Monica Molinaro, Debora Mostosi, Sabrina Saccone, Luana Tripodi, Jacopo Troisi, Annamaria Landolfi, Chiara Amoroso, Barbara Cassani, Aitor Blanco‐Míguez, Emma Leonetti, Davide Bazzani, Mattia Bolzan, Francesco Fortunato, Flavio Caprioli, Federica Facciotti, Yvan Torrente   +17 more
doaj   +2 more sources

Comparative lipidomic and metabolomic profiling of mdx and severe mdx-apolipoprotein e-null mice

Skeletal Muscle
Despite 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, Abhinandan Batra, Zoe White, David Hammers, Terence E. Ryan, Elisabeth R. Barton, Pascal Bernatchez, Glenn A. Walter   +7 more
doaj   +3 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

Low-Level Laser Therapy (LLLT) in Dystrophin-Deficient Muscle Cells: Effects on Regeneration Capacity, Inflammation Response and Oxidative Stress. [PDF]

PLoS ONE, 2015
The present study evaluated low-level laser therapy (LLLT) effects on some physiological pathways that may lead to muscle damage or regeneration capacity in dystrophin-deficient muscle cells of mdx mice, the experimental model of Duchenne muscular ...
Aline Barbosa Macedo, Luis Henrique Rapucci Moraes, Daniela Sayuri Mizobuti, Aline Reis Fogaça, Fernanda Dos Santos Rapucci Moraes, Tulio de Almeida Hermes, Adriana Pertille, Elaine Minatel   +7 more
doaj   +1 more source

Utrophin haploinsufficiency does not worsen the functional performance, resistance to eccentric contractions and force production of dystrophic mice. [PDF]

PLoS ONE, 2018
The lack of dystrophin in Duchenne muscular dystrophy (DMD) compromises the integrity and function of muscle fibers. Skeletal muscles, except the diaphragm, do not undergo progressive degeneration in adult mdx mice due to compensatory mechanisms ...
Antoine Boulanger Piette, Dounia Hamoudi, Laetitia Marcadet, Frédérique Kyomi Labelle, Rares Ovidiu David, Sabrina Bossé, Anteneh Argaw, Jérôme Frenette   +7 more
doaj   +1 more source

iNOS is not responsible for RyR1 S-nitrosylation in mdx mice with truncated dystrophin

BMC Musculoskeletal Disorders, 2020
Background Previous research indicated that nitric oxide synthase (NOS) is the key molecule for S-nitrosylation of ryanodine receptor 1 (RyR1) in DMD model mice (mdx mice) and that both neuronal NOS (nNOS) and inducible NOS (iNOS) might contribute to the
Ken’ichiro Nogami, Yusuke Maruyama, Ahmed Elhussieny, Fusako Sakai-Takemura, Jun Tanihata, Jun-ichi Kira, Yuko Miyagoe-Suzuki, Shin’ichi Takeda   +7 more
doaj   +1 more source

L-type Ca2+ channel function is linked to dystrophin expression in mammalian muscle. [PDF]

PLoS ONE, 2008
BACKGROUND: In dystrophic mdx skeletal muscle, aberrant Ca2+ homeostasis and fibre degeneration are found. The absence of dystrophin in models of Duchenne muscular dystrophy (DMD) has been connected to altered ion channel properties e.g.
Oliver Friedrich, Frederic von Wegner, Jeffrey S Chamberlain, Rainer H A Fink, Petra Rohrbach   +4 more
doaj   +1 more source

Obestatin Treatment Counteracts Muscle Wasting by Reactivation of Autophagy in Duchenne Muscular Dystrophy. [PDF]

MedComm (2020)
Obestatin signaling reactivates autophagy by NEDD4‐L activation under DMD conditions. Tyrosine switch on NEDD4‐L activates autoubiquitination that serves as a scaffold to recruit USP10 to form a deubiquitination complex, which stabilizes VPS34 to promote autophagy by activation of the Beclin1 complex. In parallel, NEDD4‐L favors AMPK exposure to CaMKKß
Santos-Zas I, Costas-Abalde S, Lodeiro AC, Fernández-Barreiro F, Cid-Díaz T, Leal-López S, González-Sánchez J, García-Lamela M, Debasa-Corral L, Mosteiro CS, Mamchaoui K, Mouly V, Casabiell X, Gallego R, Relova JL, Pazos Y, Camiña JP.   +16 more
europepmc   +2 more sources

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

Evaluation of Cardiac Function in Young Mdx Mice Using MRI with Feature Tracking and Self-Gated Magnetic Resonance Cine Imaging

Diagnostics, 2023
This study aimed to evaluate cardiac function in a young mouse model of Duchenne muscular dystrophy (mdx) using cardiac magnetic resonance imaging (MRI) with feature tracking and self-gated magnetic resonance cine imaging.
Junpei Ueda, Shigeyoshi Saito
doaj   +1 more source