RefDoc
Haut

Faire une nouvelle recherche
Make a new search
Lancer la recherche


Titre du document / Document title

The sarcolemma in the largemay mouse

Auteur(s) / Author(s)

REED Patrick W. (1) ; MATHEWS Katherine D. (2) ; MILLS Kathleen A. (2) ; BLOCH Robert J. (1) ;

Affiliation(s) du ou des auteurs / Author(s) Affiliation(s)

(1) Department of Physiology, University of Maryland School of Medicine, 660 West Redwood Street, Baltimore, Maryland 21201, ETATS-UNIS
(2) Department of Pediatrics, University of Iowa, Iowa City, Iowa, ETATS-UNIS

Résumé / Abstract

In the Largemyd mouse, dystroglycan is incompletely glycosylated and thus cannot bind its extracellular ligands, causing a muscular dystrophy that is usually lethal in early adulthood. We show that the Large-myd mutation alters the composition and organization of the sarcolemma of fast-twitch skeletal muscle fibers in young adult mice. Costameres at the sarcolemma of the tibialis anterior muscle of Largemyd mice contain reduced levels of several membrane cytoskeletal proteins, including dystrophin and β-spectrin. In the quadriceps, longitudinally oriented costameric structures tend to become thickened and branched. More strikingly, proteins of the dystrophin complex present between costameres in controls are absent from Largemyd muscles. We propose that the absence of the dystrophin complex from these regions destabilizes the sarcolemma of the Largemyd mouse and thereby contributes to the severity of its muscular dystrophy. Thus, the positioning of sarcolemmal proteins may have a profound effect on the health of skeletal muscle.

Revue / Journal Title

Muscle & nerve    ISSN  0148-639X   CODEN MUNEDE 

Source / Source

2004, vol. 30, no5, pp. 585-595 [11 page(s) (article)] (33 ref.)

Langue / Language

Anglais

Editeur / Publisher

Wiley, Hoboken, NJ, ETATS-UNIS  (1978) (Revue)

Mots-clés anglais / English Keywords

Vertebrata

;

Mammalia

;

Rodentia

;

Nervous system diseases

;

Neuromuscular diseases

;

Genetic disease

;

Sarcoglycan

;

Animal

;

Mouse

;

Striated muscle

;

Positioning

;

Muscular dystrophy

;

Quadriceps muscle

;

Membrane protein

;

Fast striated muscle

;

Muscular fiber type II

;

Organization

;

Composition

;

Mutation

;

Sarcolemma

;

Mots-clés français / French Keywords

Vertebrata

;

Mammalia

;

Rodentia

;

Système nerveux pathologie

;

Neuromusculaire pathologie

;

Maladie héréditaire

;

Sarcoglycane

;

Animal

;

Souris

;

Muscle strié

;

Positionnement

;

Dystrophie musculaire

;

Muscle quadriceps

;

Protéine membranaire

;

Muscle strié rapide

;

Fibre musculaire type II

;

Organisation

;

Composition

;

Mutation

;

Sarcolemme

;

Mots-clés espagnols / Spanish Keywords

Vertebrata

;

Mammalia

;

Rodentia

;

Sistema nervioso patología

;

Neuromuscular patología

;

Enfermedad hereditaria

;

Animal

;

Ratón

;

Músculo estriado

;

Posicionamiento

;

Distrofia muscular

;

Músculo cuadriceps

;

Proteína membranar

;

Músculo estriado rápido

;

Fibra muscular tipo II

;

Organización

;

Composicion

;

Mutación

;

Sarcolema

;

Mots-clés d'auteur / Author Keywords

costamere

;

dystroglycan

;

dystrophin

;

Largemyd

;

muscular dystrophy

;

myd

;

myodystrophy

;

sarcoglycan

;

sarcolemma

;

spectrin

;

syntrophin

;

Localisation / Location

INIST-CNRS, Cote INIST : 18909, 35400012062700.0040

Nº notice refdoc (ud4) : 16259079



Faire une nouvelle recherche
Make a new search
Lancer la recherche
Bas