Catalogo Articoli (Spogli Riviste)

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Titolo:
Parkin is linked to the ubiquitin pathway
Autore:
Tanaka, K; Suzuki, T; Chiba, T; Shimura, H; Hattori, N; Mizuno, Y;
Indirizzi:
Tokyo Metropolitan Inst Med Sci, Bunkyo Ku, Tokyo 1138613, Japan Tokyo Metropolitan Inst Med Sci Tokyo Japan 1138613 Tokyo 1138613, Japan Japan Sci & Technol Corp, CREST, Bunkyo Ku, Tokyo 1138613, Japan Japan Sci& Technol Corp Tokyo Japan 1138613 yo Ku, Tokyo 1138613, Japan Juntendo Univ, Sch Med, Dept Neurol, Bunkyo Ku, Tokyo 1130033, Japan Juntendo Univ Tokyo Japan 1130033 eurol, Bunkyo Ku, Tokyo 1130033, Japan
Titolo Testata:
JOURNAL OF MOLECULAR MEDICINE-JMM
fascicolo: 9, volume: 79, anno: 2001,
pagine: 482 - 494
SICI:
0946-2716(200109)79:9<482:PILTTU>2.0.ZU;2-V
Fonte:
ISI
Lingua:
ENG
Soggetto:
RING-FINGER PROTEINS; CARBOXY-TERMINAL HYDROLASE; F-BOX PROTEINS; ALPHA-SYNUCLEIN; JUVENILE PARKINSONISM; LIGASE ACTIVITY; CELL-DEATH; DEUBIQUITINATING ENZYMES; ANGELMAN SYNDROME; INCLUSION-BODIES;
Keywords:
parkin; autosomal recessive juvenile parkinsonism neurodegenerative disorder; Parkinson's disease; lewy body;
Tipo documento:
Review
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Life Sciences
Citazioni:
84
Recensione:
Indirizzi per estratti:
Indirizzo: Tanaka, K Tokyo Metropolitan Inst Med Sci, Bunkyo Ku, 3-18-22 Honkomagome,Tokyo 1138613, Japan Tokyo Metropolitan Inst Med Sci 3-18-22 Honkomagome Tokyo Japan 1138613
Citazione:
K. Tanaka et al., "Parkin is linked to the ubiquitin pathway", J MOL MED-J, 79(9), 2001, pp. 482-494

Abstract

Autosomal recessive juvenile parkinsonism (AR-JP) is one of the most common forms of familial Parkinson's disease. AR-JP is characterized by selective and massive loss of dopaminergic neurons in the substantia nigra of the midbrain and absence of Lewy bodies, the pathological hallmark of idiopathicParkinson's disease. Parkin, the causative gene of AR-JP, encodes a 52-kDaprotein that is a RING-type ubiquitin (Ub) protein ligase (E3) collaborating with a Ub-conjugating enzyme (E2) belonging to a cognate class of UbcH7 or UbcH8. Analysis of parkin mutations in AP-JP patients reveals that the functional loss of park-in as an E3 enzyme is the molecular basis of AR-JP. Thus it is now clear that AR-JP is due to failure of proteolysis mediated by the Ub-proteasome system and accumulation of as yet unidentified protein(s) causes nigral neuronal death without formation of Lewy bodies. These findings should shed new light on the mechanisms underlying neurodegeneration in sporadic Parkinson's disease as well as AR-JP.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 17/09/19 alle ore 23:18:42