Results 91 to 100 of about 1,087,549 (254)

MyomiRs Expression in Limb Girdle Muscular Dystrophy

IUBMB Life, Volume 77, Issue 10, October 2025.
ABSTRACT This manuscript is a comprehensive review focused on the role of microRNAs (miRs)—short RNA molecules—in Limb Girdle Muscular Dystrophy (LGMD). LGMD encompasses various and heterogeneous rare genetic neuromuscular diseases, characterized by the progressive wasting and deterioration of muscle fibers, predominantly affecting the pelvic and ...
G. Breveglieri, M. T. Altieri, M. T. Rodia, R. Costa, F. Frabetti, G. Cenacchi, G. Sabbioni, M. Borgatti   +7 more
wiley   +1 more source

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

Journal of Cachexia, Sarcopenia and Muscle, Volume 16, Issue 5, October 2025.
ABSTRACT Background Duchenne Muscular Dystrophy (DMD) features immune‐muscle crosstalk, where muscle fibre degeneration enhances pro‐inflammatory macrophage infiltration, worsening inflammation and impairing regeneration. Methods We investigated the impact of immunoproteasome (IP) inhibition on the gut‐muscle axis in mdx mice, a well‐established model ...
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
wiley   +1 more source

A mouse anti-myostatin antibody increases muscle mass and improves muscle strength and contractility in the mdx mouse model of Duchenne muscular dystrophy and its humanized equivalent, domagrozumab (PF-06252616), increases muscle volume in cynomolgus monkeys

Skeletal Muscle, 2017
BackgroundThe treatments currently approved for Duchenne muscular dystrophy (DMD), a progressive skeletal muscle wasting disease, address the needs of only a small proportion of patients resulting in an urgent need for therapies that benefit all patients
Michael St. Andre, Mark Johnson, Prashant N. Bansal, J. Wellen, A. Robertson, A. Opsahl, P. Burch, P. Bialek, C. Morris, J. Owens   +9 more
semanticscholar   +1 more source

Progressive resistance voluntary wheel running in the mdx mouse [PDF]

Muscle & Nerve, 2010
AbstractExercise training has been minimally explored as a therapy to mitigate the loss of muscle strength for individuals with Duchenne muscular dystrophy (DMD). Voluntary wheel running is known to elicit beneficial adaptations in the mdx mouse model for DMD.
James N. McKeehen, Jarrod A. Call, Susan A. Novotny, Dawn A. Lowe   +3 more
openaire   +3 more sources

ARC‐18 Improved Motor Performance Through Inhibiting ACLY‐Mediated Smad2/3 Acetylation in a Model of Duchenne Muscular Dystrophy

Journal of Cachexia, Sarcopenia and Muscle, Volume 16, Issue 5, October 2025.
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.
Chongyang Chen, Bingge Zhang, Chao Yang, Jing Wang, Ye He, Haitao Yu, Jianjun Liu, Yongmei Xie, Xifei Yang, Gong‐Ping Liu   +9 more
wiley   +1 more source

CSE/H2S/SESN2 Signalling Mediates the Protective Effect of Exercise Against Immobilization‐Induced Muscle Atrophy in Mice

Journal of Cachexia, Sarcopenia and Muscle, Volume 16, Issue 5, October 2025.
ABSTRACT Background Hydrogen sulphide (H2S), a gasotransmitter synthesized by cystathionine‐γ‐lyase (CSE), exhibits antioxidant properties and may mimic exercise‐induced muscle protection. However, its mechanistic role in muscle atrophy and exercise intervention remains unclear.
Xiuru Li, Yating Huang, Xuege Yang, Sujuan Liu, Yanmei Niu, Li Fu   +5 more
wiley   +1 more source

Reduced Muscle Force in Dystrophic DMDΔ52 Pigs Is Incompletely Restored by Systemic Transcript Reframing (DMDΔ51–52)

Journal of Cachexia, Sarcopenia and Muscle, Volume 16, Issue 5, October 2025.
ABSTRACT Background Duchenne muscular dystrophy (DMD) is a fatal X‐linked disease caused by mutations in the DMD gene, leading to dystrophin deficiency and progressive degeneration of skeletal and cardiac muscles. Pigs lacking DMD exon 52 (DMDΔ52) are a clinically severe model for DMD, mimicking molecular, functional and pathological hallmarks of the ...
Michaela Blasi, Hristiyan Hristov, Jan B. Stöckl, Martin Kraetzl, Sonja Fiedler, Elisabeth Kemter, Mayuko Kurome, Barbara Kessler, Josep M. Cambra, Valeri Zakhartchenko, Maggie C. Walter, Christian Kupatt, Nikolai Klymiuk, Thomas Fröhlich, Andreas Blutke, Michael Stirm, Florian Jaudas, Eckhard Wolf   +17 more
wiley   +1 more source

Early manifestation of alteration in cardiac function in dystrophin deficient mdx mouse using 3D CMR tagging

Journal of Cardiovascular Magnetic Resonance, 2009
Background Duchenne muscular dystrophy (DMD) is caused by the absence of the cytoskeletal protein, dystrophin. In DMD patients, dilated cardiomyopathy leading to heart failure may occur during adolescence. However, early cardiac dysfunction is frequently
Zhong Jia, Liu Wei, Li Wei, Yu Xin
doaj   +1 more source

Dystrophin isoform deficiency and upper‐limb and respiratory function in Duchenne muscular dystrophy

Developmental Medicine &Child Neurology, Volume 67, Issue 10, Page 1280-1289, October 2025.
Mary Chesshyre, Deborah Ridout, Georgia Stimpson, Valeria Ricotti, Silvana De Lucia, Erik H Niks, Volker Straub, Laurent Servais, Jean‐Yves Hogrel, Giovanni Baranello, Adnan Manzur, UK NorthStar Clinical Network and Francesco Muntoni* on behalf of the iMDEX network.
Mary Chesshyre, Deborah Ridout, Georgia Stimpson, Valeria Ricotti, Silvana De Lucia, Erik H. Niks, Volker Straub, Laurent Servais, Jean‐Yves Hogrel, Giovanni Baranello, Adnan Manzur, UK NorthStar Clinical Network, Francesco Muntoni, on behalf of the iMDEX Network, Joana Domingos, Victoria Selby, Amy Wolfe, Lianne Abbott, Efthymia Panagiotopoulou, Mario Iodice, Maria Ash, Imelda de Groot, Merel Jansen, Maaike Pelsma, Marian Bobbert, Menno Van Der Holst, Yvonne D. Krom, Marjolein J. van Heur‐Neuman, Thomas Voit, Valérie Decostre, Stéphanie Gilabert, Michela Guglieri, Alexander Murphy, Anna Mayhew, Mariacristina Scoto, Anna Sarkozy, Pinki Munot, Stephanie Robb, Elaine Chan, V Robinson, W Girshab, V Crook, E Milev, L Abbott, A Wolfe, E O’Reilly, J Watts‐When, N Burnett, R Thomas, R Terespolsky, O Martinaeu, J Longatto, C Bettolo, M Guglieri, J Diaz‐Manera, G Tasca, M Elseed, R Muni‐Lofra, M James, D Moat, J Sodhi, K Wong, E Robinson, E Groves, R Rabb, H McMurchie, H Chase, Tracey Willis, C Rylance, N Birchall, E Wright, A Childs, K Pysden, C Martos, D Roberts, L. Pallant, S Walker, A Henderson, R Madhu, R Karuvattil, Y Balla, S Gregson, S Clark, E Wraige, H Jungbluth, V Gowda, M Vanegas, J. Sheehan, A Schofield, C Smith, I Hughes, E Whitehouse, S Warner, E Reading, N Emery, J Moustoukas, K Strachan, M Ong, M Atherton, N Mills, S Sanchez Marco, A Saxena, K Skone, J TeWaterNaude, H Davis, C Wood, A Majumdar, A Murugan, I Guarino, R Tomlinson, H Jarvis, L Wills, C Frimpong, J Watson, G Cobb, G Robertson, P Brink, J Burslem, C Adams, J Wong, S Joseph, I Horrocks, J Dunne, M DiMarco, S Brown, S McKenzie, K Torne, R Mohamed, V Velmurugan, M Prasad, S Sedehizadeh, A Schugal, R Keetley, S Williamson, K Payne, E Dowling, P Fenty, C de Goede, A Parkes, K Baxter, M Illingworth, N Bhangu, S Geary, J Palmer, K Shill, S Tirupathi, A Shah, D O’Donogue, J McVeigh, J McFetridge, G Nicfhirleinn, H Beattie, T Leyland, K Stevenson, N Hussain, D Baskaran, Z Lambat, R Sullivan, L Locke, G Ambegaonkar, D Krishnakumar, J Taylor, J Moores, E Stephen, J Tewnion, S Ramdas, M Sa, A Skippen, M Khries, C Lilien, H Ramjattan, F Taylor, H English, K Stewart, F Flint, E Bartram, R Noble   +176 more
wiley   +1 more source

Misplaced Golgi Elements Produce Randomly Oriented Microtubules and Aberrant Cortical Arrays of Microtubules in Dystrophic Skeletal Muscle Fibers

Frontiers in Cell and Developmental Biology, 2019
Differentiated mammalian cells and tissues, such as skeletal muscle fibers, acquire an organization of Golgi complex and microtubules profoundly different from that in proliferating cells and still poorly understood.
Sarah Oddoux, Davide Randazzo, Aster Kenea, Bruno Alonso, Kristien J. M. Zaal, Evelyn Ralston   +5 more
doaj   +1 more source