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Titolo:
HUNTINGTIN INTERACTS WITH A FAMILY OF WW DOMAIN PROTEINS
Autore:
FABER PW; BARNES GT; SRINIDHI J; CHEN JM; GUSELLA JF; MACDONALD ME;
Indirizzi:
MASSACHUSETTS GEN HOSP E,MOL NEUROGENET UNIT,BLDG 149,13TH ST CHARLESTOWN MA 02129 MASSACHUSETTS GEN HOSP E,MOL NEUROGENET UNIT CHARLESTOWN MA 02129
Titolo Testata:
Human molecular genetics (Print)
fascicolo: 9, volume: 7, anno: 1998,
pagine: 1463 - 1474
SICI:
0964-6906(1998)7:9<1463:HIWAFO>2.0.ZU;2-X
Fonte:
ISI
Lingua:
ENG
Soggetto:
YES-ASSOCIATED PROTEIN; DISEASE GENE ALLELES; SH3 DOMAIN; SACCHAROMYCES-CEREVISIAE; 2-HYBRID SYSTEM; CAG REPEAT; BRAIN; PRODUCT; BIND; LOCALIZATION;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Science Citation Index Expanded
Citazioni:
59
Recensione:
Indirizzi per estratti:
Citazione:
P.W. Faber et al., "HUNTINGTIN INTERACTS WITH A FAMILY OF WW DOMAIN PROTEINS", Human molecular genetics (Print), 7(9), 1998, pp. 1463-1474

Abstract

The hallmark neuropathology of Huntington's disease (HD) is due to elongation of a polyglutamine segment in huntingtin, a novel similar to 350 kDa protein of unknown function. We used a yeast two-hybrid interactor screen to identify proteins whose association with huntingtin might be altered in the pathogenic process. Surprisingly, no interactors were found with internal and C-terminal segments of huntingtin, In contrast, huntingtin's N-terminus detected 13 distinct proteins, seven novel and six reported previously, Among these, we identified a major interactor class, comprising three distinct WW domain proteins, HYPA, HYPE and HYPC, that bind normal and mutant huntingtin in extracts of HD lymphoblastoid cells. This interaction is mediated by huntingtin's proline-rich region and is enhanced by lengthening the adjacent glutaminetract. Although HYPE and HYPC are novel, HYPA is human FBP-11, a protein implicated in spliceosome function, The emergence of this class ofproteins as huntingtin partners argues that a WW domain-mediated process, such as non-receptor signaling, protein degradation or pre-mRNA splicing, may participate in HD pathogenesis.

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Documento generato il 10/07/20 alle ore 19:18:54