Novel mitochondrial proteins and decreased intrinsic activity of cytochrome-C Oxidase
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Swayback disease (SD), a fatal neurological disorder affecting lambs and kids, is characterized by abnormal mitochondria and low cytochrome-c oxidase activity. The cytochrome-c oxidase content and activity and the protein composition of mitochondria isolated from the brains of SD lambs were investigated. Difference spectra analysis indicated that the cytochrome-c oxidase content of mitochondria from SD animals was lower than normal, and electrophoresis showed that when compared to mitochondria from normal animals, lipid-depleted mitochondria from SD lambs had a different protein composition, particularly, in the 40–55 kDa region. Polarographic studies, using cytochrome-c as substrate, confirmed low intrinsic activity of cytochrome-c oxidase within the mitochondria of SD lambs. These studies also showed that at low ionic strength, such mitochondria did not yield the expected characteristic biphasic Eadie-Hofstee plots.
Index EntriesSwayback mitochondria protein composition cytochrome-c oxidase content cytochrome-c oxidase activity biphasicity
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