Catalogo Articoli (Spogli Riviste)

OPAC HELP

Titolo:
Loss of huntingtin-mediated BDNF gene transcription in Huntington's disease
Autore:
Zuccato, C; Ciammola, A; Rigamonti, D; Leavitt, BR; Goffredo, D; Conti, L; MacDonald, ME; Friedlander, RM; Silani, V; Hayden, MR; Timmusk, T; Sipione, S; Cattaneo, E;
Indirizzi:
Univ Milan, Dept Pharmacol Sci, I-20133 Milan, Italy Univ Milan Milan Italy I-20133 Dept Pharmacol Sci, I-20133 Milan, Italy Univ Milan, Ctr Excellance Neurodegenerat Dis, I-20133 Milan, Italy Univ Milan Milan Italy I-20133 Neurodegenerat Dis, I-20133 Milan, Italy Univ Milan, IRCCS Osped Maggiore, Sch Med, Ctr Dino Ferrari,Dept Neurol Sci, I-20133 Milan, Italy Univ Milan Milan Italy I-20133 ari,Dept Neurol Sci, I-20133 Milan, Italy Univ British Columbia, Dept Med Genet, Vancouver, BC, Canada Univ British Columbia Vancouver BC Canada d Genet, Vancouver, BC, Canada Massachusetts Gen Hosp, Mol Neurogenet Unit, Charlestown, MA 02129 USA Massachusetts Gen Hosp Charlestown MA USA 02129 Charlestown, MA 02129 USA Harvard Univ, Brigham & Womens Hosp, Sch Med, Dept Neurosurg, Boston, MA 02115 USA Harvard Univ Boston MA USA 02115 ed, Dept Neurosurg, Boston, MA 02115 USA Univ Helsinki, Inst Biotechnol, Program Mol Neurosci, Helsinki, Finland Univ Helsinki Helsinki Finland Program Mol Neurosci, Helsinki, Finland Uppsala Univ, Ctr Biomed, Dept Dev Neurosci, Uppsala, Sweden Uppsala UnivUppsala Sweden Biomed, Dept Dev Neurosci, Uppsala, Sweden
Titolo Testata:
SCIENCE
fascicolo: 5529, volume: 293, anno: 2001,
pagine: 493 - 498
SICI:
0036-8075(20010720)293:5529<493:LOHBGT>2.0.ZU;2-W
Fonte:
ISI
Lingua:
ENG
Soggetto:
NEUROTROPHIC FACTOR; MOUSE MODEL; CELLULAR-LOCALIZATION; TARGETED DISRUPTION; GABAERGIC NEURONS; TRANSGENIC MICE; MESSENGER-RNA; RAT-BRAIN; IN-VITRO; EXPRESSION;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Agriculture,Biology & Environmental Sciences
Life Sciences
Physical, Chemical & Earth Sciences
Citazioni:
37
Recensione:
Indirizzi per estratti:
Indirizzo: Cattaneo, E Univ Milan, Dept Pharmacol Sci, Via Balzaretti 9, I-20133 Milan, Italy Univ Milan Via Balzaretti 9 Milan Italy I-20133 Milan, Italy
Citazione:
C. Zuccato et al., "Loss of huntingtin-mediated BDNF gene transcription in Huntington's disease", SCIENCE, 293(5529), 2001, pp. 493-498

Abstract

Huntingtin is a 350-kilodalton protein of unknown function that is mutatedin Huntington's disease (HD), a neurodegenerative disorder. The mutant protein is presumed to acquire a toxic gain of function that is detrimental tostriatal neurons in the brain. However, Loss of a beneficial activity of wild-type huntingtin may also cause the death of striatal neurons. Here we demonstrate that wild-type huntingtin up-regulates transcription of brain-derived neurotrophic factor (BDNF), a pro-survival factor produced by cortical neurons that is necessary for survival of striatal neurons in the brain. We show that this beneficial activity of huntingtin is Lost when the protein becomes mutated, resulting in decreased production of cortical BDNF. ThisLeads to insufficient neurotrophic support for striatal neurons, which then die. Restoring wild-type huntingtin activity and increasing BDNF production may be therapeutic approaches for treating HD.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 06/04/20 alle ore 08:43:12