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Keywords: Muscular dystrophy
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Journal Articles
Journal Articles
Journal Articles
J Cell Sci (2016) 129 (9): 1781–1791.
Published: 1 May 2016
...Noam Zuela; Monika Zwerger; Tal Levin; Ohad Medalia; Yosef Gruenbaum ABSTRACT There are roughly 14 distinct heritable autosomal dominant diseases associated with mutations in lamins A/C, including Emery–Dreifuss muscular dystrophy (EDMD). The mechanical model proposes that the lamin mutations...
Includes: Supplementary data
Journal Articles
Journal Articles
J Cell Sci (2013) 126 (12): 2678–2691.
Published: 15 June 2013
...@enders.tch.harvard.edu ) 27 03 2013 © 2013. Published by The Company of Biologists Ltd 2013 Muscle satellite cell microRNA Cell proliferation Insulin signalling Muscular dystrophy Skeletal muscle has the ability to regenerate following injury or disease. Muscle regeneration is mediated...
Includes: Supplementary data
Journal Articles
Journal Articles
J Cell Sci (2012) 125 (19): 4609–4619.
Published: 1 October 2012
... is needed for efficient myelination. * These authors contributed equally to this work ‡ Author for correspondence ( yurchenc@umdnj.edu ) 7 6 2012 © 2012. Published by The Company of Biologists Ltd 2012 Peripheral neuropathy Muscular dystrophy Basement membrane Axonal...
Includes: Supplementary data
Journal Articles
J Cell Sci (2012) 125 (17): 3957–3965.
Published: 1 September 2012
...Zhao Bo Li; Jiangyang Zhang; Kathryn R. Wagner Summary Skeletal muscle fibrosis is a defining feature of the muscular dystrophies in which contractile myofibers are replaced by fibroblasts, adipocytes and extracellular matrix. This maladaptive response of muscle to repetitive injury is progressive...
Includes: Supplementary data
Journal Articles
J Cell Sci (2012) 125 (9): 2087–2093.
Published: 1 May 2012
... . Arimura   T. , Helbling-Leclerc   A. , Massart   C. , Varnous   S. , Niel   F. , Lacène   E. , Fromes   Y. , Toussaint   M. , Mura   A. M. , Keller   D. I. , et al . ( 2005 ). Mouse model carrying H222P-Lmna mutation develops muscular dystrophy and dilated cardiomyopathy...
Includes: Supplementary data
Journal Articles
J Cell Sci (2011) 124 (21): 3654–3664.
Published: 1 November 2011
... and collagen type-I-producing cells (fibrosis) is observed in muscular dystrophies. The origin of these cells had been largely unknown, but recently we identified mesenchymal progenitors positive for platelet-derived growth factor receptor alpha (PDGFRα) as the origin of adipocytes in skeletal muscle. However...
Includes: Supplementary data
Journal Articles
Journal Articles
Journal Articles
J Cell Sci (2009) 122 (15): 2640–2650.
Published: 1 August 2009
.... Briguet, A., Courdier-Fruh, I., Foster, M., Meier, T. and Magyar, J. P. ( 2004 ). Histological parameters for the quantitative assessment of muscular dystrophy in the mdx-mouse. Neuromuscul. Disord. 14 , 675 -682. Carlsson, L., Yu, J. G. and Thornell, L. E. ( 2008 ). New aspects of obscurin...
Includes: Supplementary data
Journal Articles
J Cell Sci (2008) 121 (1): 48–54.
Published: 1 January 2008
...Marvin E. Adams; Yan Tesch; Justin M. Percival; Douglas E. Albrecht; Jay I. Conhaim; Kendra Anderson; Stanley C. Froehner α-Dystrobrevin associates with and is a homologue of dystrophin, the protein linked to Duchenne and Becker muscular dystrophies. We used a transgenic approach to restore α...
Journal Articles
Journal Articles
Journal Articles
J Cell Sci (2006) 119 (14): 2945–2952.
Published: 15 July 2006
... myopathies: wishful thinking or therapeutic perspective? J. Clin. Invest. 105 , 1669 -1674. Cossu, G. and Sampaolesi, M. ( 2004 ). New therapies for muscular dystrophy: cautious optimism. Trends Mol. Med. 10 , 516 -520. Csete, M., Walikonis, J., Slawny, N., Wei, Y., Korsnes, S., Doyle, J. C...
Journal Articles
J Cell Sci (2006) 119 (11): 2185–2195.
Published: 1 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 (2005) 118 (7): 1405–1416.
Published: 1 April 2005
... known cause of limb-girdle muscular dystrophy. Previous findings of apoptosis from γSG-deficient mice are extended here to cell culture where apoptosis is seen to increase more than tenfold in γSG-deficient myotubes compared with normal cells. The deficient myotubes also exhibit an increased contractile...