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Titolo:
Polyglutamine expansion neurodegenerative disease
Autore:
Fischbeck, KH;
Indirizzi:
NINCDS, Neurogenet Branch, NIH, Bethesda, MD 20892 USA NINCDS Bethesda MDUSA 20892 urogenet Branch, NIH, Bethesda, MD 20892 USA
Titolo Testata:
BRAIN RESEARCH BULLETIN
fascicolo: 3-4, volume: 56, anno: 2001,
pagine: 161 - 163
SICI:
0361-9230(200110/11)56:3-4<161:PEND>2.0.ZU;2-P
Fonte:
ISI
Lingua:
ENG
Soggetto:
BULBAR MUSCULAR-ATROPHY; SCA1 TRANSGENIC MICE; CREB-BINDING PROTEIN; EXPANDED POLYGLUTAMINE; INTRANUCLEAR INCLUSIONS; TRINUCLEOTIDE REPEAT; HUNTINGTONS-DISEASE; AGGREGATION; DROSOPHILA; TOXICITY;
Keywords:
neurodegeneration; Kennedy's disease; Huntington's disease; spinocerebellar ataxia; CREB-binding protein; drug screens;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
17
Recensione:
Indirizzi per estratti:
Indirizzo: Fischbeck, KH NINCDS, Neurogenet Branch, NIH, Bldg 10,Room 3B14,10 Ctr Dr,Bethesda, MD 20892 USA NINCDS Bldg 10,Room 3B14,10 Ctr Dr Bethesda MD USA 20892 USA
Citazione:
K.H. Fischbeck, "Polyglutamine expansion neurodegenerative disease", BRAIN RES B, 56(3-4), 2001, pp. 161-163

Abstract

Kennedy's disease was the first of eight neurodegenerative disorders foundto be caused by expanded polyglutamine repeats. Each of these disorders islikely caused by a toxic gain of function in the disease gene product, often associated with inclusions of mutant protein in susceptible neurons. Themechanism of toxicity may involve sequestration and depletion of a polyglutamine-containing protein that is important to neuronal survival, such as CREB-binding protein. Recent insights into the biochemistry and cellular pathology of the polyglutamine expansion neurodegenerative diseases provide the opportunity for systematic drug screens and a rational approach to effective therapy. (C) 2001 Elsevier Science Inc.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 07/04/20 alle ore 22:07:18