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Titolo:
LENGTH OF HUNTINGTIN AND ITS POLYGLUTAMINE TRACT INFLUENCES LOCALIZATION AND FREQUENCY OF INTRACELLULAR AGGREGATES
Autore:
MARTINDALE D; HACKAM A; WIECZOREK A; ELLERBY L; WELLINGTON C; MCCUTCHEON K; SINGARAJA R; KAZEMIESFARJANI P; DEVON R; KIM SU; BREDESEN DE; TUFARO F; HAYDEN MR;
Indirizzi:
UNIV BRITISH COLUMBIA,CTR MOL MED & THERAPEUT VANCOUVER BC V6T 1Z4 CANADA UNIV BRITISH COLUMBIA,CTR MOL MED & THERAPEUT VANCOUVER BC V6T 1Z4 CANADA UNIV BRITISH COLUMBIA,DEPT MICROBIOL & IMMUNOL VANCOUVER BC V6T 1Z4 CANADA UNIV BRITISH COLUMBIA,DEPT MED GENET VANCOUVER BC V6T 1Z4 CANADA UNIV BRITISH COLUMBIA,DEPT MED VANCOUVER BC V6T 1Z4 CANADA NEUROVIR INC VANCOUVER BC V6T 1Z3 CANADA BURNHAM INST,PROGRAM AGING LA JOLLA CA 92037 UNIV CALIF SAN DIEGO,DEPT NEUROSCI SAN DIEGO CA 92103 UNIV SASKATCHEWAN,COLL DENT SASKATOON SK S7N 5E4 CANADA
Titolo Testata:
Nature genetics
fascicolo: 2, volume: 18, anno: 1998,
pagine: 150 - 154
SICI:
1061-4036(1998)18:2<150:LOHAIP>2.0.ZU;2-1
Fonte:
ISI
Lingua:
ENG
Soggetto:
SIMPLEX VIRUS TYPE-1; COLORIMETRIC ASSAY; TRANSGENIC MICE; CAG REPEAT; PROTEIN; PHENOTYPE; CELLS;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Citazioni:
27
Recensione:
Indirizzi per estratti:
Citazione:
D. Martindale et al., "LENGTH OF HUNTINGTIN AND ITS POLYGLUTAMINE TRACT INFLUENCES LOCALIZATION AND FREQUENCY OF INTRACELLULAR AGGREGATES", Nature genetics, 18(2), 1998, pp. 150-154

Abstract

It is unclear how polyglutamine expansion is associated with the pathogenesis of Huntington disease (HD). Here. we provide evidence that polyglutamine expansion leads to the formation of large intracellular aggregates in vitro and in vivo. In vitro these huntingtin-containing aggregates disrupt normal cellular architecture and increase in frequency with polyglutamine length. Huntingtin truncated at nucleotide 1955, close to the caspase-3 cleavage site, forms perinuclear aggregates more readily than full-length huntingtin and increases the susceptibilityof cells to death following apoptotic stimuli. Further truncation of huntingtin to nucleotide 436 results in both intranuclear and perinuclear aggregates. For a given protein size, increasing polyglutamine length is associated with increased cellular toxicity. Asymptomatic transgenic mice expressing full-length huntingtin with 138 polyglutamines form exclusively perinuclear aggregates in neurons. These data support the hypothesis that proteolytic cleavage of mutant huntingtin leads tothe development of aggregates which compromise cell viability, and that their localization is influenced by protein length.

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Documento generato il 06/04/20 alle ore 01:52:58