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Titolo:
CASPASE CLEAVAGE OF GENE-PRODUCTS ASSOCIATED WITH TRIPLET EXPANSION DISORDERS GENERATES TRUNCATED FRAGMENTS CONTAINING THE POLYGLUTAMINE TRACT
Autore:
WELLINGTON CL; ELLERBY LM; HACKAM AS; MARGOLIS RL; TRIFIRO MA; SINGARAJA R; MCCUTCHEON K; SALVESEN GS; PROPP SS; BROMM M; ROWLAND KJ; ZHANG TQ; RASPER D; ROY S; THORNBERRY N; PINSKY L; KAKIZUKA A; ROSS CA; NICHOLSON DW; BREDESEN DE; HAYDEN MR;
Indirizzi:
UNIV BRITISH COLUMBIA,DEPT MED GENET,CTR MOL MED & THERAPEUT VANCOUVER BC V6T 1Z4 CANADA UNIV BRITISH COLUMBIA,DEPT MED GENET,CTR MOL MED & THERAPEUT VANCOUVER BC V6T 1Z4 CANADA BURNHAM INST,PROGRAM APOPTOSIS & CELL DEATH LA JOLLA CA 92037 BURNHAM INST,PROGRAM AGING LA JOLLA CA 92037 MCGILL UNIV,DEPT HUMAN GENET MONTREAL PQ CANADA MERCK FROSST CTR THERAPEUT RES,DEPT BIOCHEM & MOL BIOL MONTREAL PQ CANADA MERCK RES LABS,DEPT ENZYMOL RAHWAY NJ 00000 OSAKA BIOSCI INST,DEPT 4 OSAKA JAPAN JOHNS HOPKINS UNIV,SCH MED,DEPT NEUROSCI BALTIMORE MD 21205 JOHNS HOPKINS UNIV,SCH MED,DEPT PSYCHIAT BALTIMORE MD 21205
Titolo Testata:
The Journal of biological chemistry
fascicolo: 15, volume: 273, anno: 1998,
pagine: 9158 - 9167
SICI:
0021-9258(1998)273:15<9158:CCOGAW>2.0.ZU;2-I
Fonte:
ISI
Lingua:
ENG
Soggetto:
TRANSGENIC MICE; CAG REPEAT; CELL-DEATH; APOPTOSIS; PROTEASE; HUNTINGTIN; ACTIVATION; PHENOTYPE;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Citazioni:
36
Recensione:
Indirizzi per estratti:
Citazione:
C.L. Wellington et al., "CASPASE CLEAVAGE OF GENE-PRODUCTS ASSOCIATED WITH TRIPLET EXPANSION DISORDERS GENERATES TRUNCATED FRAGMENTS CONTAINING THE POLYGLUTAMINE TRACT", The Journal of biological chemistry, 273(15), 1998, pp. 9158-9167

Abstract

The neurodegenerative diseases Huntington disease, dentatorubropallidoluysian atrophy, spinocerebellar atrophy type 3, and spinal bulbar muscular atrophy are caused by expansion of a polyglutamine tract withintheir respective gene products. There is increasing evidence that generation of truncated proteins containing an expanded polyglutamine tract may be a key step in the pathogenesis of these disorders. We now report that, similar to huntingtin, atrophin-1, ataxin-3, and the androgen receptor are cleaved in apoptotic extracts. Furthermore, each of these proteins is cleaved by one or more purified caspases, cysteine proteases involved in apoptotic death. The CAG length does not modulate susceptibility to cleavage of any of the full-length proteins. Our results suggest that by generation of truncated polyglutamine-containing proteins, caspase cleavage may represent a common step in the pathogenesis of each of these neurodegenerative diseases.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 05/04/20 alle ore 03:41:17