Results 31 to 40 of about 25,343 (235)

Blastocyst injection of wild type embryonic stem cells induces global corrections in mdx mice. [PDF]

PLoS ONE, 2009
Duchenne muscular dystrophy (DMD) is an incurable neuromuscular degenerative disease, caused by a mutation in the dystrophin gene. Mdx mice recapitulate DMD features.
Elizabeth Stillwell, Joseph Vitale, Qingshi Zhao, Amanda Beck, Joel Schneider, Farah Khadim, Genie Elson, Aneela Altaf, Ghassan Yehia, Jia-hui Dong, Jing Liu, Willie Mark, Mantu Bhaumik, Robert Grange, Diego Fraidenraich   +14 more
doaj   +1 more source

Dystrophin gene expression and intracellular calcium changes in the giant freshwater prawn, Macrobrachium rosenbergii, in response to white spot symptom disease infection

Heliyon, 2017
Background: Dystrophin, an essential protein functional in the maintenance of muscle structural integrity is known to be responsible for muscle deterioration during white spot syndrome virus (WSSV) infection among prawn species.
Anees Fathima Noor, Tze Chiew Christie Soo, Farhana Mohd Ghani, Zee Hong Goh, Li Teng Khoo, Subha Bhassu   +5 more
doaj   +1 more source

Identification of new dystroglycan complexes in skeletal muscle. [PDF]

PLoS ONE, 2013
The dystroglycan complex contains the transmembrane protein β-dystroglycan and its interacting extracellular mucin-like protein α-dystroglycan. In skeletal muscle fibers, the dystroglycan complex plays an important structural role by linking the ...
Eric K Johnson, Bin Li, Jung Hae Yoon, Kevin M Flanigan, Paul T Martin, James Ervasti, Federica Montanaro   +6 more
doaj   +1 more source

In vivo dynamics of skeletal muscle Dystrophin in zebrafish embryos revealed by improved FRAP analysis

eLife, 2015
Dystrophin forms an essential link between sarcolemma and cytoskeleton, perturbation of which causes muscular dystrophy. We analysed Dystrophin binding dynamics in vivo for the first time.
Fernanda Bajanca, Vinicio Gonzalez-Perez, Sean J Gillespie, Cyriaque Beley, Luis Garcia, Eric Theveneau, Richard P Sear, Simon M Hughes   +7 more
doaj   +1 more source

Utilization of an Antibody Specific for Human Dystrophin to Follow Myoblast Transplantation in Nude Mice

Cell Transplantation, 1993
Human myoblasts were transplanted in nude mice. The efficacy of these transplantations was analyzed using a monoclonal antibody (NCLDys3) specific for human dystrophin. This antibody did not reveal any dystrophin in nude mice that did not receive a human
Johnny Huard, Geneviève Tremblay, Steve Verreault, Claude Labrecque, Jacques P. Tremblay   +4 more
doaj   +1 more source

Perspective: Spectrin-Like Repeats in Dystrophin Have Unique Binding Preferences for Syntrophin Adaptors That Explain the Mystery of How nNOSμ Localizes to the Sarcolemma

Frontiers in Physiology, 2018
Dystrophin is a massive multi-domain protein composed of specialized amino and carboxyl termini that are separated by 24 spectrin-like repeats. Dystrophin performs critical structural and signaling roles that are indispensable for the functional ...
Justin M. Percival
doaj   +1 more source

In vivo non-invasive monitoring of dystrophin correction in a new Duchenne muscular dystrophy reporter mouse

Nature Communications, 2019
Dystrophin-deficient mice are used to test corrective strategies for Duchenne muscular dystrophy, but evaluation of dystrophin expression requires collection of tissue samples from specific muscles and time points.
Leonela Amoasii, Hui Li, Yu Zhang, Yi-Li Min, Efrain Sanchez-Ortiz, John M. Shelton, Chengzu Long, Alex A. Mireault, Samadrita Bhattacharyya, John R. McAnally, Rhonda Bassel-Duby, Eric N. Olson   +11 more
doaj   +1 more source

Characterization of 65 epitope-specific dystrophin monoclonal antibodies in canine and murine models of duchenne muscular dystrophy by immunostaining and western blot. [PDF]

PLoS ONE, 2014
Epitope-specific monoclonal antibodies can provide unique insights for studying cellular proteins. Dystrophin is one of the largest cytoskeleton proteins encoded by 79 exons. The absence of dystrophin results in Duchenne muscular dystrophy (DMD).
Kasun Kodippili, Lauren Vince, Jin-Hong Shin, Yongping Yue, Glenn E Morris, Mark A McIntosh, Dongsheng Duan   +6 more
doaj   +1 more source

Dystrophin-Deficient Cardiomyopathy

Journal of the American College of Cardiology, 2016
Dystrophinopathies are a group of distinct neuromuscular diseases that result from mutations in the structural cytoskeletal Dystrophin gene. Dystrophinopathies include Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD), X-linked dilated cardiomyopathy, as well as DMD and BMD female carriers.
Forum, Kamdar, Daniel J, Garry
openaire   +2 more sources

When Size Really Matters: The Eccentricities of Dystrophin Transcription and the Hazards of Quantifying mRNA from Very Long Genes

Biomedicines, 2023
At 2.3 megabases in length, the dystrophin gene is enormous: transcription of a single mRNA requires approximately 16 h. Principally expressed in skeletal muscle, the dystrophin protein product protects the muscle sarcolemma against contraction-induced ...
John C. W. Hildyard, Richard J. Piercy
doaj   +1 more source