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Titolo:
Copper in disorders with neurological symptoms: Alzheimer's, Menkes, and Wilson diseases
Autore:
Strausak, D; Mercer, JFB; Dieter, HH; Stremmel, W; Multhaup, G;
Indirizzi:
Univ Heidelberg, ZMBH Ctr Mol Biol, D-69120 Heidelberg, Germany Univ Heidelberg Heidelberg Germany D-69120 , D-69120 Heidelberg, Germany Deakin Univ, Sch Biol & Chem Sci, Ctr Cellular & Mol Biol, Burwood, Vic, Australia Deakin Univ Burwood Vic Australia ar & Mol Biol, Burwood, Vic, Australia Fed Environm Agcy, Berlin, Germany Fed Environm Agcy Berlin GermanyFed Environm Agcy, Berlin, Germany Univ Heidelberg, Div Gastroenterol, Dept Med, Heidelberg, Germany Univ Heidelberg Heidelberg Germany terol, Dept Med, Heidelberg, Germany
Titolo Testata:
BRAIN RESEARCH BULLETIN
fascicolo: 2, volume: 55, anno: 2001,
pagine: 175 - 185
SICI:
0361-9230(20010515)55:2<175:CIDWNS>2.0.ZU;2-C
Fonte:
ISI
Lingua:
ENG
Soggetto:
AMYLOID-PRECURSOR-PROTEIN; WILSON-DISEASE GENE; P-TYPE ATPASE; KINKY HAIR DISEASE; C-OXIDASE DEFICIENCY; TRANS-GOLGI NETWORK; NERVE GROWTH-FACTOR; ALZHEIMER A-BETA; MENKES-DISEASE; TRANSPORTING ATPASE;
Keywords:
Alzheimer's disease; Menkes; Wilson; copper; neurodegeneration;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
170
Recensione:
Indirizzi per estratti:
Indirizzo: Multhaup, G Univ Heidelberg, ZMBH Ctr Mol Biol, Neuenheimer Feld 282, D-69120 Heidelberg, Germany Univ Heidelberg Neuenheimer Feld 282 Heidelberg Germany D-69120
Citazione:
D. Strausak et al., "Copper in disorders with neurological symptoms: Alzheimer's, Menkes, and Wilson diseases", BRAIN RES B, 55(2), 2001, pp. 175-185

Abstract

Copper is an essential element for the activity of a number of physiologically important enzymes. Enzyme-related malfunctions may contribute to severe neurological symptoms and neurological diseases: copper is a component ofcytochrome c oxidase, which catalyzes the reduction of oxygen to water, the essential step in cellular respiration. Copper is a cofactor of Cu/Zn-superoxide-dismutase which plays a key role in the cellular response to oxidative stress by scavenging reactive oxygen species. Furthermore, copper is a constituent of dopamine-p-hydroxylase, a critical enzyme in the catecholamine biosynthetic pathway. A detailed exploration of the biological importance and functional properties of proteins associated with neurological symptoms will have an important impact on understanding disease mechanisms and may accelerate development and testing of new therapeutic approaches. Copper binding proteins play important roles in the establishment and maintenance of metal-ion homeostasis, in deficiency disorders with neurological symptoms (Menkes disease, Wilson disease) and in neurodegenerative diseases (Alzheimer's disease). The Menkes and Wilson proteins have been characterized as copper transporters and the amyloid precursor protein (APP) of Alzheimer's disease has been proposed to work as a Cu(II) and/or Zn(II) transporter. Experimental, clinical and epidemiological observations in neurodegenerative disorders like Alzheimer's disease and in the genetically inherited copper-dependent disorders Menkes and Wilson disease are summarized. This could provide a rationale for a link between severely dysregulated metal-ion homeostasis and the selective neuronal pathology. (C) 2001 Elsevier Science Inc.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 04/04/20 alle ore 00:23:34