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Keywords: Dystrophin
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Journal Articles
J Cell Sci (2013) 126 (22): 5116–5131.
Published: 15 November 2013
... ( Barrero et al., 2013 ). When we combined Mef2, PCNA and dystrophin staining, we observed a large number of Mef2+ nuclei inside the myofiber ( Fig. 6A ; supplementary material Table S21 ), a few Mef2+ cells outside the myofiber that were likely to be smooth muscle cells from the vasculature ( Black...
Includes: Supplementary data
Journal Articles
J Cell Sci (2011) 124 (6): 951–957.
Published: 15 March 2011
... deletions in dystrophin. Two defined deletions predicted loss of functional spectrin-like repeats 17 and 18. Spectrin-like repeat 17 participates in actin-filament binding, and thus we hypothesized that disruption of a dystrophin–cytoplasmic actin interaction might be one of the mechanisms underlying QM...
Includes: Supplementary data
Journal Articles
J Cell Sci (2010) 123 (18): 3061–3070.
Published: 15 September 2010
...Pawan Sharma; Saeid Ghavami; Gerald L. Stelmack; Karol D. McNeill; Mark M. Mutawe; Thomas Klonisch; Helmut Unruh; Andrew J. Halayko The dystrophin–glycoprotein complex (DGC) links the extracellular matrix and actin cytoskeleton. Caveolae form membrane arrays on smooth muscle cells; we investigated...
Journal Articles
J Cell Sci (2010) 123 (12): 2008–2013.
Published: 15 June 2010
...Dejia Li; Akshay Bareja; Luke Judge; Yongping Yue; Yi Lai; Rebecca Fairclough; Kay E. Davies; Jeffrey S. Chamberlain; Dongsheng Duan Duchenne muscular dystrophy (DMD) is a lethal muscle disease caused by dystrophin deficiency. In normal muscle, dystrophin helps maintain sarcolemmal stability...
Journal Articles
J Cell Sci (2007) 120 (22): 3999–4008.
Published: 15 November 2007
..., as well as increased plasma creatine kinase levels. Costameres at the sarcolemma of keratin-19-null muscle, visualized with antibodies against spectrin or dystrophin, were disrupted and the sarcolemma was separated from adjacent myofibrils by a large gap in which mitochondria accumulated. The costameric...
Journal Articles
J Cell Sci (2007) 120 (6): 996–1008.
Published: 15 March 2007
...Angela K. Peter; Gaynor Miller; Rachelle H. Crosbie The dystrophin-glycoprotein complex spans the muscle plasma membrane and provides a mechanical linkage between laminin in the extracellular matrix and actin in the intracellular cytoskeleton. Within the dystrophin-glycoprotein complex...
Journal Articles
J Cell Sci (2006) 119 (13): 2679–2687.
Published: 01 July 2006
...Toshifumi Yokota; Qi-Long Lu; Jennifer E. Morgan; Kay E. Davies; Rosie Fisher; Shin'ichi Takeda; Terence A. Partridge Duchenne muscular dystrophy and the mdx mouse myopathies reflect a lack of dystrophin in muscles. However, both contain sporadic clusters of revertant fibers (RFs) that express...
Journal Articles
J Cell Sci (2006) 119 (11): 2185–2195.
Published: 01 June 2006
...Jachinta E. Rooney; Jennifer V. Welser; Melissa A. Dechert; Nichole L. Flintoff-Dye; Stephen J. Kaufman; Dean J. Burkin The dystrophin glycoprotein complex links laminin in the extracellular matrix to the cell cytoskeleton. Loss of dystrophin causes Duchenne muscular dystrophy, the most common...
Includes: Multimedia, Supplementary data
Journal Articles
J Cell Sci (2006) 119 (8): 1537–1546.
Published: 15 April 2006
...Luke M. Judge; Miki Haraguchiln; Jeffrey S. Chamberlain Duchenne muscular dystrophy is a severe disorder caused by mutations in the dystrophin gene. Dystrophin is required for assembly of the dystrophin-glycoprotein complex and provides a mechanically strong link between the cytoskeleton...
Journal Articles
J Cell Sci (2004) 117 (17): 3821–3830.
Published: 01 August 2004
...Michael J. Allikian; Andrew A. Hack; Stephanie Mewborn; Ulrike Mayer; Elizabeth M. McNally Disruption of the sarcoglycan complex leads to muscle membrane instability and muscular dystrophy in humans and mice. Through the dystrophin glycoprotein complex, sarcoglycan participates in connecting...
Includes: Multimedia, Supplementary data
Journal Articles
J Cell Sci (2001) 114 (11): 2065–2076.
Published: 01 June 2001
... address: Department of Human Genetics M1-159, Academic Medical Center, University of Amsterdam, Meibergdreef 9, 1105 AZ Amsterdam, The Netherlands 9 3 2001 © The Company of Biologists Limited 2001 2001 Plectin Dystrophin Actin-binding domain Focal contact Plectin is a high...