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Titolo:
EXCESSIVE FORMATION OF HYDROXYL RADICALS AND ALDEHYDIC LIPID-PEROXIDATION PRODUCTS IN CULTURED SKIN FIBROBLASTS FROM PATIENTS WITH COMPLEX-I DEFICIENCY
Autore:
LUO XP; PITKANEN S; KASSOVSKABRATINOVA S; ROBINSON BH; LEHOTAY DC;
Indirizzi:
HOSP SICK CHILDREN,RES INST,DIV CLIN BIOCHEM,DEPT PEDIAT LAB MED,555 UNIV AVE TORONTO ON M5G 1X8 CANADA UNIV TORONTO,DEPT PEDIAT LAB MED,DIV CLIN BIOCHEM TORONTO ON CANADA UNIV TORONTO,DEPT BIOCHEM TORONTO ON CANADA HOSP SICK CHILDREN,RES INST,DEPT GENET TORONTO ON M5G 1X8 CANADA
Titolo Testata:
The Journal of clinical investigation
fascicolo: 12, volume: 99, anno: 1997,
pagine: 2877 - 2882
SICI:
0021-9738(1997)99:12<2877:EFOHRA>2.0.ZU;2-6
Fonte:
ISI
Lingua:
ENG
Soggetto:
MITOCHONDRIAL ELECTRON-TRANSPORT; CONGENITAL CATARACT; LACTIC-ACIDOSIS; HYPERTROPHIC CARDIOMYOPATHY; PYRUVATE-DEHYDROGENASE; NADH DEHYDROGENASE; ENDOTHELIAL-CELLS; OXIDATIVE STRESS; SUPEROXIDE ANION; GENERATION;
Keywords:
COMPLEX I DEFICIENCY; CARDIOMYOPATHY; CATARACTS; HYDROXYL RADICALS; LIPID PEROXIDATION;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Citazioni:
51
Recensione:
Indirizzi per estratti:
Citazione:
X.P. Luo et al., "EXCESSIVE FORMATION OF HYDROXYL RADICALS AND ALDEHYDIC LIPID-PEROXIDATION PRODUCTS IN CULTURED SKIN FIBROBLASTS FROM PATIENTS WITH COMPLEX-I DEFICIENCY", The Journal of clinical investigation, 99(12), 1997, pp. 2877-2882

Abstract

Previous studies suggest oxygen free radicals' involvement in the etiology of cardiomyopathy with cataracts. To investigate the role of free radicals in the pathogenesis of the cardiomyopathy with cataracts and complex I deficiency, fibroblasts from patients were assessed for hydroxyl radical formation and aldehydic lipid peroxidation products with and without redox active agents that increase free radicals. The rate of hydroxyl radical formation in patient cells was increased over 2-10-fold under basal conditions, and up to 20-fold after menadione or doxorubicin treatment compared with normal cells. We also found an overproduction of aldehydes in patient cells both under basal conditions and after treatment. Both hydroxyl radicals and toxic aldehydes such ashexanal, 4-hydroxynon-2-enal, and malondialdehyde were elevated in cells from patients with three types of complex I deficiency. In contrast, acyloins, the less toxic conjugated products of pyruvate and saturated aldehydes, were lower in the patient cells. Our data provide direct evidence for the first time that complex I deficiency is associated with excessive production of hydroxyl radicals and lipid peroxidation. The resultant damage may contribute to the early onset of cardiomyopathy and cataracts and death in early infancy in affected patients withthis disease.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 30/05/20 alle ore 15:00:58