Results 21 to 30 of about 23,636 (243)

Effect of beta-Dystroglycan Processing on Utrophin / DP116 Anchorage in Normal and MDX Mouse Schwann Cell Membrane [PDF]

Neuroscience 141 (18/04/2006) 607-620, 2007
In the peripheral nervous system, utrophin and the short dystrophin isoform (Dp116) are co-localized at the outermost layer of the myelin sheath of nerve fibers; together with the dystroglycan complex. In peripheral nerve, matrix metalloproteinase (MMP) creates a 30 kDa fragment of beta-dystroglycan, leading to a disruption of the link between the ...
arxiv   +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

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

Low dystrophin variability between muscles and stable expression over time in Becker muscular dystrophy using capillary Western immunoassay

Scientific Reports, 2021
Becker muscular dystrophy (BMD) is the milder allelic variant of Duchenne muscular dystrophy, with higher dystrophin levels. To anticipate on results of interventions targeting dystrophin expression it is important to know the natural variation of ...
Z. Koeks, A. A. Janson, C. Beekman, M. Signorelli, H. A. van Duyvenvoorde, J. C. van den Bergen, M. T. Hooijmans, I. Alleman, I. M. Hegeman, J. J. G. M. Verschuuren, J. C. v. Deutekom, P. Spitali, N. A. Datson, E. H. Niks   +13 more
doaj   +1 more source

Larger interface area at the human myotendinous junction in type 1 compared with type 2 muscle fibers

Scandinavian Journal of Medicine &Science in Sports, Volume 33, Issue 2, Page 136-145, February 2023., 2023
The myotendinous junction (MTJ) is structurally specialized to transmit force. The highly folded muscle membrane at the MTJ increases the contact area between muscle and tendon and potentially the load tolerance of the MTJ. Muscles with a high content of type II fibers are more often subject to strain injury compared with muscles with type I fibers. It
Jens Rithamer Jakobsen, Abigail Louise Mackey, Manuel Koch, Thomas Imhof, Jens Hannibal, Michael Kjær, Michael Rindom Krogsgaard   +6 more
wiley   +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

Current knowledge of dystrophin and dystrophin-associated proteins in the retina.

Histology and histopathology, 2000
Dramatical development of molecular genetics has been disclosing the molecular mechanism of Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD). DMD gene product, dystrophin, is a submembranous cytoskeletal protein and many dystrophin-associated proteins (DAPs) have been identified, such as utrophin, dystroglycans, sarcoglycans ...
Ueda, H., Baba, T., Ohno, S.
openaire   +4 more sources

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

Dystrophins in vertebrates and invertebrates [PDF]

Human Molecular Genetics, 1998
Members of the dystrophin family of proteins perform a critical but incompletely characterized role in the maintenance of membrane-associated complexes at points of intercellular contact in many vertebrate cell types. They interact with, amongst others, the transmembrane laminin receptor dystroglycan, cytoskeletal actin and, indirectly, the ...
Roland G. Roberts, Martin Bobrow
openaire   +2 more sources