Results 31 to 40 of about 1,104,554 (244)

How much dystrophin is enough: the physiological consequences of different levels of dystrophin in the mdx mouse [PDF]

Human Molecular Genetics, 2015
Splice modulation therapy has shown great clinical promise in Duchenne muscular dystrophy, resulting in the production of dystrophin protein. Despite this, the relationship between restoring dystrophin to established dystrophic muscle and its ability to ...
Betts, C, Coursindel, T, El Andaloussi, S, Gait, M J, Godfrey, C, Hammond, S, Hildyard, J C W, McClorey, G, Muses, S, O'Donovan, L, Terry, R L, Wells, D J, Wells, K E, Wood, M J   +13 more
core   +3 more sources

Preventing phosphorylation of dystroglycan ameliorates the dystrophic phenotype in mdx mouse [PDF]

, 2012
Loss of dystrophin protein due to mutations in the DMD gene causes Duchenne muscular dystrophy. Dystrophin loss also leads to the loss of the dystrophin glycoprotein complex (DGC) from the sarcolemma which contributes to the dystrophic phenotype ...
Andrew Mitchell, Andrä, Assereto, Batchelor, Bonuccelli, Bonuccelli, Chris J. Moore, Clerk, Cluchague, Cox, Deconinck, Dominic J. Wells, Ervasti, Ervasti, Ervasti, Foster, Gaynor Miller, Gazzerro, Goyenvalle, Grady, Greenberg, Gundesli, Hoyte, Hu, Ilsley, James, Judge, Karpati, Konieczny, Lipscomb, Matsumura, McArthur, Miller, Moore, Morgan, Nguyen, Otte, Pereboev, Pichavant, Pulkkinen, Rebecca Terry, Rezniczek, Robert Piggott, Schröder, Sharp, Smith, Sotgia, Sotgia, Steve J. Winder, T. Neil Dear, Thompson, Thompson, Tinsley, Tinsley, Tracy La Riviere, Winder   +55 more
core   +4 more sources

IL-6 signaling blockade increases inflammation but does not affect muscle function in the mdx mouse [PDF]

BMC Musculoskeletal Disorders, 2012
Background IL-6 is a pleiotropic cytokine that modulates inflammatory responses and plays critical roles in muscle maintenance and remodeling. In the mouse model (mdx) of Duchenne Muscular Dystrophy, IL-6 and muscle inflammation are elevated, which is ...
Kostek Matthew C, Nagaraju Kanneboyina, Pistilli Emidio, Sali Arpana, Lai San-Huei, Gordon Brad, Chen Yi-Wen   +6 more
doaj   +2 more sources

Dystrophin expression in the mdx mouse restored by stem cell transplantation [PDF]

Nature, 1999
E. Gussoni, Y. Soneoka, Corinne D. Strickland, E. Buzney, Mohamed K. Khan, A. Flint, L. Kunkel, R. Mulligan   +7 more
semanticscholar   +2 more sources

Six weeks of N-acetylcysteine antioxidant in drinking water decreases pathological fiber branching in MDX mouse dystrophic fast-twitch skeletal muscle. [PDF]

Front Physiol, 2023
Redwan A, Kiriaev L, Kueh S, Morley JW, Houweling P, Perry BD, Head SI.   +6 more
europepmc   +3 more sources

Macrophages escape Klotho gene silencing in the mdx mouse model of Duchenne muscular dystrophy and promote muscle growth and increase satellite cell numbers through a Klotho-mediated pathway [PDF]

Human Molecular Genetics, 2018
Michelle Wehling-Henricks, Steven S. Welc, Guiseppina Samengo, C. Rinaldi, C. Lindsey, Ying Wang, Jeongyoon Lee, M. Kuro-o, J. Tidball   +8 more
semanticscholar   +2 more sources

"Of Mice and Measures": A Project to Improve How We Advance Duchenne Muscular Dystrophy Therapies to the Clinic [PDF]

, 2018
A new line of dystrophic mdx mice on the DBA/2J (D2) background has emerged as a candidate to study the efficacy of therapeutic approaches for Duchenne muscular dystrophy (DMD). These mice harbor genetic polymorphisms that appear to increase the severity
Aartsma-Rus, A., Bogdanik, L, Davies, K, De Luca, A, Demonbruen, Ar, Duan, D, Elsey, D, Fukada, Si, Girgenrath, M, Gonzalez, Jp, Gordish-Dressman, H, Grounds, Md, Heydemann, A, Kreibich, A, Lutz, C, Nagaraju, K, Nichols, A, Partridge, T, Passini, M, Pazze, Ld, Sanarica, F, Schnell, Fj, Spencer, M, Spurney, C, van Putten, M, Wells, Dj, Willmann, R, Yokota, T, Young, Cs, Zhong, Z   +29 more
core   +3 more sources

Wild-type mouse models to screen antisense oligonucleotides for exon-skipping efficacy in Duchenne muscular dystrophy. [PDF]

PLoS ONE, 2014
A readily available animal model is essential for rapidly identifying effective treatments for Duchenne muscular dystrophy (DMD), a devastating neuromuscular disorder caused by the lack of dystrophin protein, which results from frame-disrupting mutations
Limin Cao, Gang Han, Ben Gu, HaiFang Yin
doaj   +1 more source

Hematopoietic Prostaglandin D Synthase Inhibitor PK007 Decreases Muscle Necrosis in DMD mdx Model Mice

Life, 2021
Duchenne muscular dystrophy (DMD) is characterized by progressive muscle weakness and wasting due to the lack of dystrophin protein. The acute phase of DMD is characterized by muscle necrosis and increased levels of the pro-inflammatory mediator ...
Sai Yarlagadda, Christina Kulis, Peter G. Noakes, Mark L. Smythe   +3 more
doaj   +1 more source

Nintedanib decreases muscle fibrosis and improves muscle function in a murine model of dystrophinopathy [PDF]

, 2018
Duchenne muscle dystrophy (DMD) is a genetic disorder characterized by progressive skeletal muscle weakness. Dystrophin deficiency induces instability of the sarcolemma during muscle contraction that leads to muscle necrosis and replacement of muscle by ...
Díaz Manera, Jordi, Escudero Cuadrado, Luis María, Fernández Simón, Esther, Gallardo, Eduard, Gómez Gálvez, Pedro, Piñol Jurado, Patricia, Suárez Calvet, Xavier   +6 more
core   +1 more source