Mitochondrial changes associated with glutathione deficiency
Autor: | Alton Meister |
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Rok vydání: | 1995 |
Předmět: |
GPX1
GPX3 Antimetabolites Guinea Pigs Glutathione reductase Mitochondria Liver Ascorbic Acid Mitochondrion Biology GPX4 Models Biological Mice chemistry.chemical_compound Methionine Sulfoximine Buthionine sulfoxide Animals Ascorbate Buthionine sulfoximine Buthionine Sulfoximine Molecular Biology Cerebral Cortex chemistry.chemical_classification Reactive oxygen species Glutathione deficiency Glutathione Mitochondria Rats Animals Newborn Liver chemistry Biochemistry Molecular Medicine |
Zdroj: | Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease. 1271:35-42 |
ISSN: | 0925-4439 |
DOI: | 10.1016/0925-4439(95)00007-q |
Popis: | Glutathione deficiency produced by giving buthionine sulfoximine (an inhibitor of gamma-glutamylcysteine synthetase) to animals, leads to biphasic decline in cellular glutathione levels associated with sequestration of glutathione in mitochondria. Liver mitochondria lack the enzymes needed for glutathione synthesis. Mitochondrial glutathione arises from the cytosol. Rat liver mitochondria have a multicomponent system (with Kms of approx. 60 microM and 5.4 mM) that underlies their remarkable ability to transport and retain glutathione. Mitochondria produce substantial quantities of reactive oxygen species; this is opposed by reactions involving glutathione. Glutathione deficiency leads to widespread mitochondrial damage which is lethal in newborn rats and guinea pigs, animals that do not synthesize ascorbate. Glutathione esters and ascorbate protect against the lethal and other effects of glutathione deficiency. Ascorbate spares glutathione; it increases mitochondrial glutathione in glutathione-deficient animals. Glutathione esters delay onset of scurvy in ascorbate-deficient guinea pigs; thus, glutathione spares ascorbate. Glutathione and ascorbate function together in protecting mitochondria from oxidative damage. |
Databáze: | OpenAIRE |
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