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Titolo:
Structural comparison of actin binding in utrophin and dystrophin
Autore:
Keep, NH;
Indirizzi:
Univ London Birkbeck Coll, BBSRC Struct Biol Ctr, London WC1E 7HX, EnglandUniv London Birkbeck Coll London England WC1E 7HX ndon WC1E 7HX, England Univ London Birkbeck Coll, Sch Crystallog, London WC1E 7HX, England Univ London Birkbeck Coll London England WC1E 7HX ndon WC1E 7HX, England
Titolo Testata:
NEUROLOGICAL SCIENCES
fascicolo: 5, volume: 21, anno: 2000, supplemento:, S
pagine: S929 - S937
SICI:
1590-1874(2000)21:5<S929:SCOABI>2.0.ZU;2-0
Fonte:
ISI
Lingua:
ENG
Soggetto:
BECKER MUSCULAR-DYSTROPHY; CALPONIN HOMOLOGY DOMAIN; F-ACTIN; ALPHA-ACTININ; CROSS-LINKING; ROD DOMAIN; BETA-DYSTROGLYCAN; CRYSTAL-STRUCTURE; TERMINAL REGION; SODIUM-CHANNELS;
Keywords:
dystrophin; muscular dystrophies; utrophin;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Citazioni:
78
Recensione:
Indirizzi per estratti:
Indirizzo: Keep, NH Univ London Birkbeck Coll, BBSRC Struct Biol Ctr, Malet St, London WC1E 7HX, England Univ London Birkbeck Coll Malet St London England WC1E7HX gland
Citazione:
N.H. Keep, "Structural comparison of actin binding in utrophin and dystrophin", NEUROL SCI, 21(5), 2000, pp. S929-S937

Abstract

Dystrophin links the actin cytoskeleton to the dystroglycan complex in theplasma membrane as part of the linkage between the cytoskeleton and the extracellular matrix. Damage to or absence of dystrophin causes Duchenne or Becker muscular dystrophy. It has been proposed that elevating the levels ofutrophin, a close homologue of dystrophin, may act as a therapy for these forms of muscular dystrophy. This requires that there is a close functionalequivalence of these two proteins. In both utrophin and dystrophin, the main actin-binding region is at. the N terminus. It is related to sequences found in a number of other proteins including alpha -actinin, spectrin and fimbrin. Recent structural and biochemical studies of these proteins have shown that although the method of binding to actin is broadly similar, there are significant differences. There are even differences between utrophin and dystrophin. These studies imply that some caution should be applied to claims that utrophin and dystrophin are completely functionally interchangeable. In this paper, I review studies that elucidate and compare the actin-binding function of utrophin and dystrophin, particularly those initiated in the laboratory of Dr. John Kendrick-Jones at the MRC in Cambridge.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 29/03/20 alle ore 08:09:54