Titre du document / Document title

The sarcolemma in the large^may 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 Large^myd 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 Large^myd 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 Large^myd muscles. We propose that the absence of the dystrophin complex from these regions destabilizes the sarcolemma of the Large^myd 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, n^o5, pp. 585-595 [11 page(s) (article)] (33 ref.)

Langue / Language

Anglais

Editeur / Publisher

Wiley, New York, NY, 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