Results 31 to 40 of about 7,866 (244)

Increased circulating levels of interleukin-6 induce perturbation in redox-regulated signaling cascades in muscle of dystrophic mice [PDF]

, 2017
Duchenne muscular dystrophy (DMD) is an X-linked genetic disease in which dystrophin gene is mutated, resulting in dysfunctional or absent dystrophin protein.
Forcina, Laura, Musaro', Antonio, Nicoletti, Carmine, Pelosi, Laura, Scicchitano, Bianca Maria   +4 more
core   +3 more sources

Biglycan meets utrophin [PDF]

Science-Business eXchange, 2011
Tivorsan Pharmaceuticals is working with a Brown University team to optimize recombinant biglycan for delivery to patients with Duchenne muscular dystrophy. The work builds on the researchers' finding that the extracellular matrix glycoprotein decreases muscle pathology and improves muscle function in dystrophic mice.
openaire   +1 more source

Clinical and genetic characterisation of dystrophin-deficient muscular dystrophy in a family of Miniature Poodle dogs [PDF]

, 2018
Four full-sibling intact male Miniature Poodles were evaluated at 4–19 months of age. One was clinically normal and three were affected. All affected dogs were reluctant to exercise and had generalised muscle atrophy, a stiff gait and a markedly elevated
A Aartsma-Rus, A McKenna, Alberta de Stefani, Anita Shea, AP Monaco, AV Winnard, BA Valentine, BF Smith, BJ Cooper, C Le Guiner, C Pichavant, CA Collins, CA Jenkins, CA Wetterman, Carlos E. Ambrósio, CT Caskey, DE Cole, DJ VanBelzen, Elsa Beltrán, EM Hoogerwaard, EM Hoogerwaard, EP Hoffman, F Daoud, G Anil, G Bulfield, G. Diane Shelton, GD Shelton, GD Shelton, GD Shelton, GD Shelton, GL Walmsley, H Li, JA Goldstein, JL Carpenter, JN Kornegay, JN Kornegay, JR Mendell, JW McGreevy, K Bushby, K Nakamura, KJ Livak, KJ Nowak, Ling T. Guo, Lluís Sánchez, Louise M. Burmeister, LT Guo, Luisa De Risio, M Koenig, N Klymiuk, N Winand, NJ Olby, NJ Sharp, PP Nghiem, S Atencia-Fernandez, S Pouwels, S Rozen, SJ Schatzberg, SJ Schatzberg, T Larcher, U Francke, V Dubowitz, V Ricotti, W El Nemer, WI Baltzer   +63 more
core   +3 more sources

Micro-dystrophin gene therapy demonstrates long-term cardiac efficacy in a severe Duchenne muscular dystrophy model

Molecular Therapy: Methods & Clinical Development, 2023
Micro-dystrophin gene replacement therapies for Duchenne muscular dystrophy (DMD) are currently in clinical trials, but have not been thoroughly investigated for their efficacy on cardiomyopathy progression to heart failure. We previously validated Fiona/
Arden B. Piepho, Jeovanna Lowe, Laurel R. Cumby, Lisa E. Dorn, Dana M. Lake, Neha Rastogi, Megan D. Gertzen, Sarah L. Sturgill, Guy L. Odom, Mark T. Ziolo, Federica Accornero, Jeffrey S. Chamberlain, Jill A. Rafael-Fortney   +12 more
doaj   +1 more source

Revertant fibres and dystrophin traces in Duchenne muscular dystrophy: Implication for clinical trials [PDF]

, 2010
Duchenne muscular dystrophy (DMD) is characterised by the absence of dystrophin in muscle biopsies, although residual dystrophin can be present, either as dystrophin-positive (revertant) fibres or traces.
Arechavala-Gomeza, V, Bushby, K, Edge, G, Feng, L, Guglieri, M, Hunt, D, Kinali, M, Lehovsky, J, Main, M, Morgan, JE, Muntoni, F, Sewry, CA, Straub, V   +12 more
core   +1 more source

Utrophin influences mitochondrial pathology and oxidative stress in dystrophic muscle

Skeletal Muscle, 2017
Background Duchenne muscular dystrophy (DMD) is a lethal X-linked muscle wasting disorder caused by the absence of dystrophin, a large cytoskeletal muscle protein.
Tahnee L. Kennedy, Lee Moir, Sarah Hemming, Ben Edwards, Sarah Squire, Kay Davies, Simon Guiraud   +6 more
doaj   +1 more source

PMO-based let-7c site blocking oligonucleotide (SBO) mediated utrophin upregulation in mdx mice, a therapeutic approach for Duchenne muscular dystrophy (DMD)

Scientific Reports, 2020
Upregulation of utrophin, a dystrophin related protein, is considered a promising therapeutic approach for Duchenne muscular dystrophy (DMD). Utrophin expression is repressed at the post-transcriptional level by a set of miRNAs, among which let-7c is ...
Kasturi Sengupta, Emanuele Loro, Tejvir S. Khurana   +2 more
doaj   +1 more source

Inactivation of Sirt6 ameliorates muscular dystrophy in mdx mice by releasing suppression of utrophin expression

Nature Communications, 2022
Utrophin is a dystrophin-related protein stabilizing the sarcolemma in absence of dystrophin. Here the authors report that inactivation of the protein deacetylase SIRT6, involved in the deacetylation of the epigenetic mark H3K56ac in muscle cells ...
Angelina M. Georgieva, Xinyue Guo, Marek Bartkuhn, Stefan Günther, Carsten Künne, Christian Smolka, Ann Atzberger, Ulrich Gärtner, Kamel Mamchaoui, Eva Bober, Yonggang Zhou, Xuejun Yuan, Thomas Braun   +12 more
doaj   +1 more source

Sertoli Cells Improve Myogenic Differentiation, Reduce Fibrogenic Markers, and Induce Utrophin Expression in Human DMD Myoblasts

Biomolecules, 2021
Duchenne muscular dystrophy (DMD) is an X-linked disease caused by mutations in DMD gene translating in lack of functional dystrophin and resulting in susceptibility of myofibers to rupture during contraction.
Laura Salvadori, Sara Chiappalupi, Iva Arato, Francesca Mancuso, Mario Calvitti, Maria Cristina Marchetti, Francesca Riuzzi, Riccardo Calafiore, Giovanni Luca, Guglielmo Sorci   +9 more
doaj   +1 more source

Thermodynamic stability, unfolding kinetics, and aggregation of the N-terminal actin-binding domains of utrophin and dystrophin. [PDF]

, 2012
Muscular dystrophy (MD) is the most common genetic lethal disorder in children. Mutations in dystrophin trigger the most common form of MD, Duchenne, and its allelic variant Becker MD.
Aaartsma-Rus, Ahmad, Badorff, Barghorn, Belli, Blake, Blake, Chamberlain, Chenna, Chi, Cotton, Courdier-Fruh, Daragan, Deconinck, Deconinck, Delaglio, Deol, Ebihara, Emery, Ervasti, Espargaró, Fairclough, Fisher, Foster, Galkin, Galkin, Gilbert, Gimona, Grady, Gramolini, Greenfield, Gregorevic, Gregorevic, Hamed, Helliwell, Henderson, Henderson, Hoffman, Ishikawa-Sakurai, Ito, Kahana, Karpati, Keep, Khurana, Khurana, Kim, Klunk, Knuesel, Krishna, Lakowicz, Le Rumeur, Legardinier, Lehman, Levine-III, Li, Lin, Love, Love, Mallela, Matsumura, Mayer, Mirza, Miura, Moores, Muir, Muntoni, Myers, Nagarajan, Norwood, Odom, Odom, Ohlendieck, Orlova, Park, Parsell, Pastoret, Pearce, Phelps, Pons, Prior, Prior, Prochniewicz, Rafael, Rafael, Roberts, Roberts, Roberts, Ruschak, Ruszczak, Rybakova, Rybakova, Santoro, Santoro, Scott, Shatsky, Sieb, Singh, Singh, Sitnik, Sonnemann, Squire, Sutherland-Smith, Tanabe, Tinsley, Tinsley, Tinsley, van Deutekom, Wakefield, Wang, Wang, Winder, Winder   +111 more
core   +1 more source