PMID: 9429156Jan 16, 1998Paper

The membrane-cytoskeleton interface: the role of dystrophin and utrophin

Journal of Muscle Research and Cell Motility
S J Winder

Abstract

Recent studies with transgenic animals have considerably advanced our knowledge of the roles of dystrophin and utrophin in both muscle and non-muscle tissues. Rigorous analyses of the roles of the various mdx mutations in mice, as well as the use of artificial transgenes in an mdx background, are beginning to define the functional importance of various regions of the dystrophin protein in normal muscle. Furthermore, recent biochemical analyses have revealed new insights into the role and organization of dystrophin at the membrane-cytoskeleton interface. Transgenic approaches have also revealed surprising and encouraging results with respect to utrophin. Against expectations, the long-awaited utrophin knockout mice have a remarkably mild phenotype with only subtle changes in neuromuscular junction architecture. On the other hand, mdx mice transgenic for a mini-utrophin construct showed rescue of the muscular dystrophy phenotype, clearly an encouraging finding with obvious therapeutic possibilities. These and other recent findings are discussed in the context of the structure and function of dystrophin and utrophin at the membrane-cytoskeleton interface.

Citations

Aug 26, 1999·Journal of Neurochemistry·M T Moral-NaranjoC J Vidal
Aug 3, 2000·Neuroreport·H Rosas-VargasB Cisneros
Jul 13, 2000·Cell Motility and the Cytoskeleton·C A Moores, J Kendrick-Jones
Jan 14, 2004·FASEB Journal : Official Publication of the Federation of American Societies for Experimental Biology·Ashok KumarAladin M Boriek
Jun 25, 2009·Genes to Cells : Devoted to Molecular & Cellular Mechanisms·Maki Masuda-HirataShigeo Ohno
Jun 18, 2011·Molecular Biology of the Cell·Cathy SirourNicole Moreau
Mar 13, 1999·The Journal of Biological Chemistry·R BettoG Salviati
Apr 11, 2000·Microscopy Research and Technique·A O GramoliniB J Jasmin
Feb 12, 2011·Journal of Muscle Research and Cell Motility·K P García-PelagioH González-Serratos
Jan 10, 2012·Journal of Biomedicine & Biotechnology·Richard A ZuelligMarcus C Schaub
Dec 14, 1999·Journal of Microscopy· De Eguileor MG Lanzavecchia
Jan 1, 2003·BioEssays : News and Reviews in Molecular, Cellular and Developmental Biology·Joe V Chakkalakal, Bernard J Jasmin
Jan 9, 2008·Blood Coagulation & Fibrinolysis : an International Journal in Haemostasis and Thrombosis·Doris CerecedoDalila Martínez-Rojas
Jul 14, 2010·Journal of Biomedicine & Biotechnology·Caroline Perronnet, Cyrille Vaillend
Oct 26, 2012·Journal of Orthopaedic Research : Official Publication of the Orthopaedic Research Society·Christian IsaacJohnny Huard
Feb 19, 2010·Cell Communication and Signaling : CCS·Chris J Moore, Steve J Winder
Jul 27, 1999·Neuropathology and Applied Neurobiology·A A Hassoni, M J Cullen
Mar 26, 2002·FEBS Letters·Kristina Djinovic-CarugoPaul Young
Nov 26, 2015·Expert Opinion on Orphan Drugs·Julian Ramos, Jeffrey S Chamberlain
Nov 26, 1998·Cell Motility and the Cytoskeleton·B A RenleyJ M Ervasti
Jul 12, 2002·BioEssays : News and Reviews in Molecular, Cellular and Developmental Biology·Heather J SpenceSteven J Winder
Dec 17, 2004·Journal of Cell Science·Enrique O Hernández-GonzálezDalila Martínez-Rojas
Jan 29, 2002·Cellular Signalling·Jane L IlsleySteven J Winder
Aug 26, 1998·Biochemical and Biophysical Research Communications·D M FinnK Ohlendieck
Jun 18, 2002·Journal of Structural Biology·Michael J F Broderick, Steven J Winder
Jun 23, 2001·Biochimica Et Biophysica Acta·A V PereboevG E Morris
Feb 13, 2001·Trends in Biochemical Sciences·S J Winder
Sep 11, 2002·Neuromuscular Disorders : NMD·Kelly J Perkins, Kay E Davies
Nov 14, 2002·Journal of Agricultural and Food Chemistry·Halldor Sigfusson, Herbert O Hultin

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