PMID: 7516752Mar 1, 1994Paper

Expression of dystrophin-associated proteins in dystrophin-positive muscle fibers (revertants) in Duchenne muscular dystrophy

Neuromuscular Disorders : NMD
K MatsumuraK P Campbell

Abstract

The dystrophin-glycoprotein complex spans the sarcolemma to provide a linkage between the subsarcolemmal cytoskeleton and the extracellular matrix in skeletal muscle. In Duchenne muscular dystrophy (DMD), the absence of dystrophin leads to a drastic reduction in all of the dystrophin-associated proteins in the sarcolemma, thus causing the disruption of the dystrophin-glycoprotein complex and the loss of the linkage to the extracellular matrix. This is presumed to lead to sarcolemmal instability which could render muscle fibers susceptible to necrosis. In DMD, a very small percentage of muscle fibers show dystrophin staining along the sarcolemma, presumably due to a second in-frame deletion in the dystrophin gene. However, the functional significance of these rare dystrophin-positive muscle fibers (revertants) in DMD has been unclear. Here we report the co-expression of the dystrophin-associated proteins with dystrophin in revertants of DMD skeletal muscle. Our results suggest that the entire dystrophin-glycoprotein complex is restored in revertants and, thus, the linkage between the subsarcolemmal cytoskeleton and the extracellular matrix is restored in these muscle fibers.

References

Dec 10, 1992·Nature·J M TinsleyD R Love
Aug 1, 1992·The Journal of Clinical Investigation·R D BiesR Fenwick
Feb 1, 1992·The Journal of Clinical Investigation·D RécanL V Nicholson
Dec 1, 1991·The Journal of Cell Biology·K Ohlendieck, K P Campbell
Sep 20, 1991·Cell·J M Ervasti, K P Campbell
Jan 1, 1991·The Journal of Cell Biology·K OhlendieckK P Campbell
Jun 3, 1991·FEBS Letters·K Ohlendieck, K P Campbell
Oct 1, 1990·Journal of the Neurological Sciences·E P HoffmanT A Partridge
Mar 16, 1989·Nature·K P Campbell, S D Kahl
Dec 1, 1989·Journal of the Neurological Sciences·L V NicholsonJ B Harris

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Citations

Mar 28, 2003·Biochemical and Biophysical Research Communications·Stephen P ChambersDonald R Love
Aug 18, 1999·Journal of the Neurological Sciences·M Fanin, C Angelini
Feb 17, 2000·Trends in Neurosciences·D J Blake, S Kröger
Oct 6, 2000·Comptes rendus de l'Académie des sciences. Série III, Sciences de la vie·E ChaubourtS de La Porte
Jan 5, 2002·Journal of Physiology, Paris·Emmanuel ChaubourtSabine De La Porte
Mar 21, 2001·Neuromuscular Disorders : NMD·L PolitanoG Nigro
Oct 26, 2002·Neuromuscular Disorders : NMD·Sigalit LeibovitzUri Nudel
Dec 7, 2002·Neuromuscular Disorders : NMD·Emily V HowmanKay E Davies
Dec 1, 1996·Neuromuscular Disorders : NMD·D J Duggan, E P Hoffman
Dec 1, 1996·Neuromuscular Disorders : NMD·M JeanpierreJ C Kaplan
Jan 1, 1997·Neuromuscular Disorders : NMD·K MatsumuraT Shimizu
Feb 3, 1998·Neuromuscular Disorders : NMD·K E Davies
May 1, 1997·Neuromuscular Disorders : NMD·H IshiiK Arahata
Aug 26, 1998·Neuromuscular Disorders : NMD·C A Sewry
Jul 10, 1999·Neuromuscular Disorders : NMD·N HattoriT Hazama
Mar 14, 2000·Neuromuscular Disorders : NMD·S DecaryG S Butler-Browne
May 7, 2009·Proceedings of the National Academy of Sciences of the United States of America·Jachinta E RooneyDean J Burkin
Aug 30, 1994·Proceedings of the National Academy of Sciences of the United States of America·J M TinsleyK E Davies
Apr 1, 1996·Australian and New Zealand Journal of Medicine·Z RudzkiJ V Lloyd
Oct 3, 2013·International Journal of Molecular Sciences·Daniela ErriquezAlessandra Ferlini
Jul 5, 2012·Comparative and Functional Genomics·Chiara Lanzuolo
Sep 5, 2009·The American Journal of Pathology·Jennifer V WelserDean J Burkin
Dec 3, 2014·The Journal of Thoracic and Cardiovascular Surgery·Philipp TreskesYeong-Hoon Choi
Mar 18, 2011·Journal of Proteome Research·Laetitia GuevelLynn A Megeney
Feb 16, 2021·Frontiers in Cell and Developmental Biology·Hongmei LuoBo Zuo
Jun 8, 2014·Disease Models & Mechanisms·Chunhui JiangShihuan Kuang
Jun 1, 1996·Seminars in Pediatric Neurology·B Reitter, H H Goebel
Jan 30, 2007·Pharmacology & Therapeutics·Gordon S LynchJames G Ryall

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