Catalogo Articoli (Spogli Riviste)

OPAC HELP

Titolo:
EXPRESSION OF DELETION-CONTAINING DYSTROPHINS IN MDX MUSCLE - IMPLICATIONS FOR GENE-THERAPY AND DYSTROPHIN FUNCTION
Autore:
FRITZ JD; DANKO I; ROBERDS SL; CAMPBELL KP; LATENDRESSE JS; WOLFF JA;
Indirizzi:
UNIV WISCONSIN,WAISMAN CTR,DEPT PEDIAT,ROOM 355,1500 HIGHLAND AVE MADISON WI 53705 UNIV WISCONSIN,WAISMAN CTR,DEPT PEDIAT MADISON WI 53705 UNIV WISCONSIN,WAISMAN CTR,DEPT MED GENET MADISON WI 53705 UNIV IOWA,COLL MED,HOWARD HUGHES MED INST IOWA CITY IA 52242 UNIV IOWA,COLL MED,DEPT PHYSIOL & BIOPHYS IOWA CITY IA 52242
Titolo Testata:
Pediatric research
fascicolo: 6, volume: 37, anno: 1995,
pagine: 693 - 700
SICI:
0031-3998(1995)37:6<693:EODDIM>2.0.ZU;2-7
Fonte:
ISI
Lingua:
ENG
Soggetto:
DUCHENNE MUSCULAR-DYSTROPHY; ADENOVIRUS-MEDIATED TRANSFER; COOH-TERMINAL DOMAINS; CYSTEINE-RICH DOMAIN; SKELETAL-MUSCLE; GLYCOPROTEIN COMPLEX; MINIDYSTROPHIN GENE; MOUSE; MICE; PROTEINS;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Citazioni:
45
Recensione:
Indirizzi per estratti:
Citazione:
J.D. Fritz et al., "EXPRESSION OF DELETION-CONTAINING DYSTROPHINS IN MDX MUSCLE - IMPLICATIONS FOR GENE-THERAPY AND DYSTROPHIN FUNCTION", Pediatric research, 37(6), 1995, pp. 693-700

Abstract

The expression of full-length dystrophin and various dystrophin deletion mutants was monitored in mdx mouse muscle after intramuscular injection of dystrophin-encoding plasmid DNAs. Recombinant dystrophin proteins, including those lacking either the amino terminus, carboxyl terminus, or most of the central rod domain, showed localization to the plasma membrane. This suggests that there are multiple attachment sites for dystrophin to the plasma membrane. Only those constructs containing the carboxyl terminus were able to stabilize dystrophin-associated proteins (DAP) at the membrane, consistent with other studies that suggest that this domain is critical to DAP binding. Colocalization with DAP was not necessary for membrane localization of the various dystrophin molecules. However, stabilization and co-localization of the DAP did seem to be a prerequisite for expression and/or stabilization of mutant dystrophins beyond 1 wk and these same criteria seemed important for mitigating the histopathological consequences of dystrophin deficiency.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 02/12/20 alle ore 07:30:27