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Titolo:
DIFFERENTIAL INHIBITION BY HYPERAMMONEMIA OF THE ELECTRON-TRANSPORT CHAIN ENZYMES IN SYNAPTOSOMES AND NONSYNAPTIC MITOCHONDRIA IN ORNITHINETRANSCARBAMPLASE-DEFICIENT SPF-MICE - RESTORATION BY ACETYL-L-CARNITINE
Autore:
QURESHI K; RAO KVR; QURESHI IA;
Indirizzi:
UNIV MONTREAL,DIV MED GENET,HOP ST JUSTINE,DEPT PEDIAT,3175 COTE ST CATHERINE MONTREAL PQ H3T 1C5 CANADA
Titolo Testata:
Neurochemical research
fascicolo: 6, volume: 23, anno: 1998,
pagine: 855 - 861
SICI:
0364-3190(1998)23:6<855:DIBHOT>2.0.ZU;2-R
Fonte:
ISI
Lingua:
ENG
Soggetto:
ACUTE AMMONIA INTOXICATION; ALZHEIMERS-DISEASE; ENERGY-METABOLISM; CONGENITAL HYPERAMMONEMIA; CYTOCHROME-OXIDASE; BINDING-SITES; BRAIN; RATS; NA+,K+-ATPASE; DEHYDROGENASE;
Keywords:
ORNITHINE TRANSCARBAMYLASE DEFICIENCY; BRAIN; SYNAPTOSOMES; NONSYNAPTIC MITOCHONDRIA; ELECTRON TRANSPORT CHAIN; ACETYL-L-CARNITINE;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Science Citation Index Expanded
Citazioni:
37
Recensione:
Indirizzi per estratti:
Citazione:
K. Qureshi et al., "DIFFERENTIAL INHIBITION BY HYPERAMMONEMIA OF THE ELECTRON-TRANSPORT CHAIN ENZYMES IN SYNAPTOSOMES AND NONSYNAPTIC MITOCHONDRIA IN ORNITHINETRANSCARBAMPLASE-DEFICIENT SPF-MICE - RESTORATION BY ACETYL-L-CARNITINE", Neurochemical research, 23(6), 1998, pp. 855-861

Abstract

Sparse-fur (spf) mouse is the ideal animal model to study the neuropathology of congenital ornithine transcarbamylase (OTC) deficiency. Ourcurrent hypothesis implies that an ammonia-induced depletion of energy metabolism in the spf mouse, could be due to a reduction in the activities of the enzymes of the electron transport chain and a treatment with acetyl-L-carnitine could normalize this abnormality. We also hypothesized that there might be a differential degree of inhibition in synaptosomal and non-synaptic mitochondria, for the enzymes of the electron transport chain, caused by congenital hyperammonemia. We have therefore measured the activities of NADH-cytochrome C oxidoreductase, succinate cytochrome C oxidoreductase and cytochrome C oxidase in synaptosomes and non-synaptic mitochondria, isolated from spf mice and CD-1 controls with and without acetyl-L-carnitine treatment. Our results indicate a significant reduction (19-34%) in the activities of these complexes in synaptosomes in untreated spf mice, whereas in non-synaptic mitochondria, there was a tendency for the activities to decrease, Acetyl-L-carnitine treatment enhanced these activities (15-64%) for all the three enzyme complexes and its effect was more prominent on succinate cytochrome C oxidoreductase activity (64%). These studies point out that: (a) ammonia-induced disturbances in the energy metabolism could be more pronounced in neuronal mitochondria, and (b) the effect of acetyl-L-carnitine on the restoration of cerebral ATP in hyperammonemia could be through an enhancement of the activities of various electron transport chain enzymes.

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Documento generato il 28/01/20 alle ore 21:07:58