Results 11 to 20 of about 37,729 (256)

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   +3 more sources

Estrogen-Related Receptor Alpha Promotes Skeletal Muscle Regeneration and Mitigates Muscular Dystrophy. [PDF]

FASEB J
ERRα promotes muscle regeneration. ERRα drives angiogenic and mitochondrial metabolic gene program in proliferating and differentiating myogenic cells. ERRα also induces myogenic factor genes such as MyoG. Through these pathways ERRα promotes muscle regeneration in the skeletal muscle in acute injury and chronic myopathy.
Nguyen TTH, Huang YX, Poliakova S, Citu C, Mann E, Sopariwala DH, Zhao Z, Kumar A, Narkar VA.   +8 more
europepmc   +2 more sources

ARC-18 Improved Motor Performance Through Inhibiting ACLY-Mediated Smad2/3 Acetylation in a Model of Duchenne Muscular Dystrophy. [PDF]

J Cachexia Sarcopenia Muscle
ABSTRACT Background Duchenne muscular dystrophy (DMD) is a genetic disorder characterized by progressive muscle weakness, with inflammation and fibrosis contributing to its pathogenesis. Despite advancements in genetic disease‐modifying treatment, there is currently no effective pharmacological treatment for DMD.
Chen C, Zhang B, Yang C, Wang J, He Y, Yu H, Liu J, Xie Y, Yang X, Liu GP.   +9 more
europepmc   +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

Improvement of Spontaneous Locomotor Activity in a Murine Model of Duchenne Muscular Dystrophy by N-Acetylglucosamine Alone and in Combination With Prednisolone. [PDF]

FASEB J
Spontaneous locomotor activity of Duchenne muscular dystrophy (DMD) model mice was monitored using a cage system with external sensors. Mice received oral United State Pharmacopeia‐grade N‐acetylglucosamine (GlcNAc), prednisolone (Pred)—a current standard of care for DMD—or a combination of both.
Satoh MS, St-Pierre G, Rancourt A, Fillion M, Sato S.   +4 more
europepmc   +2 more sources

From fibro/adipogenic progenitors to adipocytes: Understanding adipogenesis in muscle degeneration for disease modulation. [PDF]

J Physiol
Abstract figure legend Fibro/adipogenic progenitors (FAPs) are cells resident in the muscle (skeletal and cardiac) niche. FAPs are active participants in the process of muscle degeneration in cardiovascular and neuromuscular diseases. Here, the accumulation of fatty and fibrous tissue is a hallmark.
Villalobos E, Mehra P, Diaz-Manera J.
europepmc   +2 more sources

Stress exposure in the mdx mouse model of Duchenne muscular dystrophy provokes a widespread metabolic response. [PDF]

FEBS J
Duchenne muscular dystrophy is a severe neuromuscular wasting disease that is caused by a primary defect in dystrophin protein. A targeted mass‐spectrometry‐based metabolomics assay was conducted to identify the impact of stress exposure on the regulation of biological stress pathways in the mdx mouse model of Duchenne muscular dystrophy.
Johnson EE, Ervasti JM.
europepmc   +2 more sources

Oestrogen Receptor Alpha in Myocyte Maintains Muscle Regeneration in Duchenne Muscular Dystrophy [PDF]

Journal of Cachexia, Sarcopenia and Muscle
Background Oestrogen receptor alpha (ERα) plays an important role in maintaining mitochondrial function and regulating metabolism in skeletal muscle.
Xiaofei Huang, Sijia Li, Huna Wang, Lei Zhao, Xihua Li, Shusheng Fan, Wanting Hu, Haowei Tong, Guangyao Guo, Dengqiu Xu, Luyong Zhang, Zhenzhou Jiang, Qinwei Yu   +12 more
doaj   +2 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