PMID: 1822352Jan 1, 1991Paper

Expression of myosin heavy chain isoforms in Duchenne muscular dystrophy patients and carriers

Neuromuscular Disorders : NMD
J F MariniJ J Léger

Abstract

The expression of MHC isoforms in the skeletal muscles of nine patients with Duchenne muscular dystrophy (DMD) (from 2.5 to 15 yr of age) and three DMD carriers was studied using different specific anti-MHC MAbs. We also analyzed muscle fiber size and fiber reactivity with acridine orange and/or with a surface antigen marker. One-quarter of all fibers of DMD patients, or less with age, were of normal size and contained only adult slow MHC. Half of the muscle fibers contained adult and developmental MHCs. Only half of these fibers were representative of an active regenerative process. MHC co-expression also altered the proportion of normal fast or slow fibers. Adult fast MHCs were expressed as unique MHC only in small and very small fibers in the oldest DMD patients. In DMD carrier muscles, the greatest alterations in MHC expression were observed in patients with the most reduced dystrophin expression. However, MHC changes in dystrophin-positive fibers were similar to those observed in dystrophin-free fibers. In conclusion, disruptions or delays in the switching of all genes coding for adult fast and slow MHC and developmental MHC coincided with dystrophin deletion and with perturbations in its expression.

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Citations

May 15, 2010·Cell and Tissue Research·Michael Kottlors, Janbernd Kirschner
Nov 2, 1999·Neuromuscular Disorders : NMD·B L PattonJ R Sanes
Mar 14, 2000·Neuromuscular Disorders : NMD·S DecaryG S Butler-Browne
Oct 12, 2013·Histopathology·Landry-Cyrille BankoléFawzi Kadi
Dec 13, 2005·The Journal of Physiology·Giuseppe D'AntonaRoberto Bottinelli
Jun 7, 2014·Frontiers in Aging Neuroscience·Rüdiger RudolfMichael R Deschenes
Sep 16, 2014·Frontiers in Aging Neuroscience·Kunihiro SakumaAkihiko Yamaguchi
Dec 19, 1998·The Journal of Histochemistry and Cytochemistry : Official Journal of the Histochemistry Society·Z Yablonka-ReuveniA J Rivera
Aug 11, 2007·Muscle & Nerve·Paul GregorevicJeffrey S Chamberlain
Jan 1, 1996·Biochemistry and Cell Biology = Biochimie Et Biologie Cellulaire·A N PernitskyJ E Anderson
Jan 21, 2000·Journal of Applied Physiology·B CormeryP F Gardiner
May 12, 2019·European Archives of Oto-rhino-laryngology : Official Journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS) : Affiliated with the German Society for Oto-Rhino-Laryngology - Head and Neck Surgery·Kumiko SekikawaMasahiro Itoh
Jul 5, 2016·Expert Review of Proteomics·Paul DowlingKay Ohlendieck
Sep 24, 2021·Pflügers Archiv : European journal of physiology·Kay Ohlendieck, Dieter Swandulla

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