Results 21 to 30 of about 28,350 (246)

Pathological evaluation of rats carrying in-frame mutations in the dystrophin gene: a new model of Becker muscular dystrophy

Disease Models & Mechanisms, 2020
Dystrophin, encoded by the DMD gene on the X chromosome, stabilizes the sarcolemma by linking the actin cytoskeleton with the dystrophin-glycoprotein complex (DGC).
Naomi Teramoto, Hidetoshi Sugihara, Keitaro Yamanouchi, Katsuyuki Nakamura, Koichi Kimura, Tomoko Okano, Takanori Shiga, Taku Shirakawa, Masafumi Matsuo, Tetsuya Nagata, Masao Daimon, Takashi Matsuwaki, Masugi Nishihara   +12 more
doaj   +1 more source

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

Function and Genetics of Dystrophin and Dystrophin-Related Proteins in Muscle [PDF]

Physiological Reviews, 2002
The X-linked muscle-wasting disease Duchenne muscular dystrophy is caused by mutations in the gene encoding dystrophin. There is currently no effective treatment for the disease; however, the complex molecular pathology of this disorder is now being unravelled.
Blake, D, Weir, A, Newey, S, Davies, K
openaire   +2 more sources

Biological role of dystroglycan in Schwann cell function and its implications in peripheral nervous system diseases [PDF]

, 2010
Dystroglycan is a central component of the dystrophin-glycoprotein complex (DGC) that links extracellular matrix with cytoskeleton, expressed in a variety of fetal and adult tissues.
Kiichiro Matsumura, Matsumura, Kiichiro, Masaki, Toshihiro, Toshihiro Masaki   +3 more
core   +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

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

Alterations in dihydropyridine receptors in dystrophin-deficient cardiac muscle [PDF]

, 2006
The deficiency of dystrophin, a critical membrane stabilizing protein, in the mdx mouse causes an elevation in intracellular calcium in myocytes. One mechanism that could elicit increases in intracellular calcium is enhanced influx via the L-type calcium
Lu, Sai, Watson, M, Woolf, Peter James, Hoey, AJ, Cornford-Nairn, R, Holroyd, SM, Hoey, Andrew, Woolf, PJ, Cornford-Nairn, Renee, Hoey, Andrew J., Xiao, Xiao-Hui, Woolf, Peter J., Xiao, XH, Holroyd, Sean M., Watson, Michael, Cornford-Nairn, Renee A., Brown, Lindsay, Holroyd, Sean, Brown, L   +18 more
core   +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

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