The sarcolemma in the largemay mouse

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

Localisation / Location

INIST-CNRS, Cote INIST : 18909, 35400012062700.0040

Nº notice refdoc (ud4) : 16259079