Copper Deficiency in the Rat. Effect on Adenine Nucleotide Metabolism

Gallagher, C. H.; Reeve, Vivienne E.

doi:10.1038/icb.1971.48

Cited by 17 publications

(6 citation statements)

References 8 publications

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“…It is, however, more likely that defective cytochrome oxidase activity in the brain is responsible for much of the cerebral pathology, in particular the cerebellar lesion and the myelin pallor noted in all cases. This would be in accord with the findings of Gallagher and Reeve (1971) of deficient phospholipid synthesis in the brains of copper-deficient rats.…”

supporting

confidence: 90%

Therapeutic implications of copper deficiency in Menkes's steely-hair syndrome.

Walker-Smith¹,

Turner²,

Blomfield³

et al. 1973

Archives of Disease in Childhood

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supporting

confidence: 90%

Therapeutic implications of copper deficiency in Menkes's steely-hair syndrome.

Walker-Smith¹,

Turner²,

Blomfield³

et al. 1973

Archives of Disease in Childhood

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“…This degree of involvement could be explained by deficiency of a trace element which plays a r81e in a basic enzymic process, and several studies point to such a conclusion. Gallagher and Reeve (1971) found that phospholipid synthesis is defective in the brains of copper-deficient rats. French et a/.…”

Section: Discussionmentioning

confidence: 99%

Neuropathology of Menkes' Disease

Barnard

Best

Erdohazi

1978

Develop Med Child Neuro

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SUMMARY Three new unrelated cases of Menkes' disease are recorded. All three were male infants and died between six and 14 months of age. In each case the clinical diagnosis was confirmed by low serum levels of copper and by examination of the abnormal hair. Treatment did not affect the progress of the disease. Neuropathological findings were widespread cortical neuronal loss, demyelination and gliosis of the white matter and profound reduction of all elements of the cerebellum. Other features were meningo‐encephalitis, subdural haematoma, and ventricular enlargement due to aqueductal gliosis. The findings are comparable with those in eight other recorded cases reviewed in the present study. The pattern of the disease indicates impaired antenatal and postnatal development, complicated by subsequent degeneration of the central nervous system. These processes are believed to be the result of the underlying metabolic disorder. RÉSUMÉ La neuropathologie de la maladie de Menkes Trois cas non apparentés et nouveaux de maladie de Menkes sont présentés. Tous les trois étaient des garçons morts entre six et 14 mois. Dans chaque cas, le diagnostic clinique fut confirmé par les faibles taux sériques du cuivre et par l'examen des cheveux anormaux. Le traitement n'avait pas modifié l'évolution de la maladie. L'examen neuropathologique révéla des pertes diffuses de neurones corticaux, une démyélinisation et une gliose de la substance blanche, une réduction importante de tous les éléments du cervelet. Les autres caractéristiques étaient une méningo‐encéphalite, un hématome sous‐dural et une dilatation ventriculaire par gliose de l'aqueduc. Ces donnees neuropathologiques sont comparables à celles trouvées dans les huit autres cas revus de l'étude présente. L'allure de la maladie indique un développement anté‐natal et post‐natal altéré compliqué par une dégénération ultérieure du système nerveux central. On peut penser que ces processus sont le résultat d'un trouble métabolique sousjacent. ZUSAMMENFASSUNG Neuropathologische Befunde der Menkes' Krankheit Es wird über drei neue, nicht verwandte Fälle mit der Menkes' Krankheit berichtet. Alle drei Kinder waren männlichen Geschlechtes und starben im Alter von sechs bis 14 Monaten. Bei jedem Patienten wurde die Diagnose durch niedrige Serum‐Kupfer‐Spiegel und durch Untersuchung des abnormen Haares bestätigt. Eine Behandlung verhinderte das Fortschreiten der Erkrankung nicht. Neuropathologisch fand sich ein ausgedehnter Verlust von corticalen Neuronen, Demyelinisierung und Gliose der weissen Substanz und eine ausgeprägte Verminderung aller Strukturen im Kleinhirn. Andere charakteristische Merkmale waren Meningo‐Encephalitis, subdurale Hämatome und Ventrikelvergrösserung auf Grund einer Gliose im Aquaeduktbereich. Die neuropathologischen Befunde waren im Einklang mit denen von acht anderen Patienten, über die zusammenfassend in der vorliegenden Arbeit berichtet wird. Die Symptome weisen auf eine gestörte prae‐ und postnatale Entwicklung hin, die durch die daraus resultierende Degenerati...

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“…However, a relationship between reduced cytochrome-c oxidase activity and defective energy metabolism in causing the pathophysiological consequences associated with copper deficiency has not been clearly established. For instance, a 70%-90% reduction in cytochrome-c oxidase activity during copper deficiency either has no effect or only modestly lowers brain, liver, and platelet ATP content (5)(6)(7)(8)(9). Thus, if reduced cytochrome-c oxidase activity contributes to the biological consequences of copper deficiency, then an alternative to impaired energy production as a mechanism needs to be found.…”

mentioning

confidence: 99%

Copper Deprivation Potentiates Oxidative Stress in HL‐60 Cell Mitochondria

Johnson

Thomas

1999

Proc Soc Exp Biol Med

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Cytochrome-c oxidase is the copper-dependent terminal respiratory complex (complex IV) of the mitochondrial electron transport chain whose activity in a variety of tissues is lowered by copper deficiency. Because inhibition of respiratory complexes increases the production of reactive oxygen species by mitochondria, it is possible that copper deficiency increases oxidative stress in mitochondria as a consequence of suppressed cytochrome-c oxidase activity. In this study, the activities of respiratory complex I + III, assayed as NADH:cytochrome-c reductase, complex II + III, assayed as succinate:cytochrome-c reductase, complex IV, assayed as cytochrome-c oxidase, and fumarase were measured in mitochondria from HL-60 cells that were grown for seven passages in serum-free medium that was either unsupplemented or supplemented with 50 n M CuSO4. Fumarase activity was not affected by copper supplementation, but the complex I + III:fumarase and complex IV:fumarase ratios were reduced 30% and 50%, respectively, in mitochondria from cells grown in the absence of supplemental copper. This indicates that copper deprivation suppressed the electron transfer activity of copper-independent complex I + III as well as copper-dependent complex IV. Manganese superoxide dismutase (MnSOD) content was also increased 49% overall in the cells grown in the absence of supplemental copper. Furthermore, protein carbonyl groups, indicative of oxidative modification, were present in 100-kDa and 90-kDa proteins of mitochondria from copper-deprived cells. These findings indicate that in cells grown under conditions of copper deprivation that suppress cytochrome-c oxidase activity, oxidative stress in mitochondria is increased sufficiently to induce MnSOD, potentiate protein oxidation, and possibly cause the oxidative inactivation of complex I.

show abstract

Copper Deficiency in the Rat. Effect on Adenine Nucleotide Metabolism

Cited by 17 publications

References 8 publications

Therapeutic implications of copper deficiency in Menkes's steely-hair syndrome.

Therapeutic implications of copper deficiency in Menkes's steely-hair syndrome.

Neuropathology of Menkes' Disease

Copper Deprivation Potentiates Oxidative Stress in HL‐60 Cell Mitochondria

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