Novel mitochondrial proteins and decreased intrinsic activity of cytochrome-c oxidase. Characteristics of swayback disease in sheep.
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.
Alleyne
T
T
Department of Biochemistry, Faculty of Medical Sciences, University of the West Indies, St. Augustine, Trinidad and Tobago.
Adogwa
A
A
Lalla
A
A
Joseph
J
J
John
R
R
D000818:Animals; D001921:Brain / Q000378:metabolism*; D003576:Electron Transport Complex IV / Q000378:metabolism*; D008928:Mitochondria / Q000378:metabolism*; D010084:Oxidation-Reduction; D011048:Polarography; D011506:Proteins / Q000302:isolation & purification / Q000378:metabolism*; D012016:Reference Values; D012756:Sheep; D013540:Swayback / Q000378:metabolism*
D011506:Proteins; D003576:Electron Transport Complex IV