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Titolo:
Huntingtin: an iron-regulated protein essential for normal nuclear and perinuclear organelles
Autore:
Hilditch-Maguire, P; Trettel, F; Passani, LA; Auerbach, A; Persichetti, F; MacDonald, ME;
Indirizzi:
Massachusetts Gen Hosp, Mol Neurogenet Lab, Charlestown, MA 02129 USA Massachusetts Gen Hosp Charlestown MA USA 02129 Charlestown, MA 02129 USA NYU, Sch Med, Howard Hughes Med Inst, New York, NY 10016 USA NYU New YorkNY USA 10016 Howard Hughes Med Inst, New York, NY 10016 USA NYU, Sch Med, Skirball Inst Biomol Med, Dept Cell Biol, New York, NY 10016USA NYU New York NY USA 10016 omol Med, Dept Cell Biol, New York, NY 10016USA
Titolo Testata:
HUMAN MOLECULAR GENETICS
fascicolo: 19, volume: 9, anno: 2000,
pagine: 2789 - 2797
SICI:
0964-6906(20001122)9:19<2789:HAIPEF>2.0.ZU;2-G
Fonte:
ISI
Lingua:
ENG
Soggetto:
DISEASE GENE-PRODUCT; EMBRYONIC LETHALITY; MUTANT HUNTINGTIN; CELL-LINES; WILD-TYPE; IN-VIVO; LOCALIZATION; HOMOLOG; BRAIN; IDENTIFICATION;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
40
Recensione:
Indirizzi per estratti:
Indirizzo: MacDonald, ME Massachusetts Gen Hosp, Mol Neurogenet Lab, Bldg 149,13th St, Charlestown,MA 02129 USA Massachusetts Gen Hosp Bldg 149,13th St Charlestown MA USA 02129
Citazione:
P. Hilditch-Maguire et al., "Huntingtin: an iron-regulated protein essential for normal nuclear and perinuclear organelles", HUM MOL GEN, 9(19), 2000, pp. 2789-2797

Abstract

Huntington's; disease (HD), with its selective neuronal cell loss, is caused by an elongated glutamine tract in the huntingtin protein, To discover the pathways that are candidates for the protein's normal and/or abnormal function, we surveyed 19 classes of organelle in Hdh(ex4/5)/Hdh(ex4/5) knock-out compared with wild-type embryonic stem cells to identify any that mightbe affected by huntingtin deficiency, Although the majority did not differ, dramatic changes in six classes revealed that huntingtin's function is essential for the normal nuclear (nucleoli, transcription factor-speckles) and perinuclear membrane (mitochondria, endoplasmic reticulum, Golgi and recycling endosomes) organelles and for proper regulation of the iron pathway. Moreover, upmodulation by deferoxamine mesylate implicates huntingtin as aniron-response protein, However, excess huntingtin produced abnormal organelles that resemble the deficiency phenotype, suggesting the importance of huntingtin level to the protein's normal pathway, Thus, organelles that require huntingtin to function suggest roles for the protein in RNA biogenesis,trafficking and iron homeostasis to be explored in HD pathogenesis.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 19/09/20 alle ore 12:35:41