Depletion of brain mitochondria cytochrome oxidase in the mottled mouse mutant.
The mouse mutant Mobr is an animal model of Menkes kinky hair syndrome with a similar defect in copper utilization. The copper-dependent enzyme, cytochrome oxidase from the brain, liver, and heart mitochondria was examined. The brain and heart from Mobr/y had significantly less cytochrome alpha + alpha 3 than normal animals' when the cytochrome absorption spectra of tissue samples from animals 11 to 13 days of age were analyzed. Liver cytochrome was not significantly different. When brain mitochondrial cytochrome oxidase spectrograms from animals of different ages were examined, a major change was found to occur during the 2nd week of life. When cytochrome oxidase activity from brain mitochondria was measured, assaying the rate of oxidation of cytochrome c, the results were similar to those from spectrogram analysis.
D000818:Animals; D001921:Brain / Q000201:enzymology* / Q000648:ultrastructure; D003300:Copper / Q000378:metabolism; D003576:Electron Transport Complex IV / Q000378:metabolism*; D007706:Menkes Kinky Hair Syndrome / Q000378:metabolism; D051379:Mice; D008817:Mice, Mutant Strains / Q000378:metabolism*; D008928:Mitochondria / Q000201:enzymology*; D008929:Mitochondria, Heart / Q000201:enzymology; D008930:Mitochondria, Liver / Q000201:enzymology; D000623:Porphobilinogen Synthase / Q000378:metabolism
D003300:Copper; D003576:Electron Transport Complex IV; D000623:Porphobilinogen Synthase