Novel mitochondrial proteins and decreased intrinsic activity of cytochrome-c oxidase. Characteristics of swayback disease in sheep

Mol Chem Neuropathol. May-Aug 1996;28(1-3):285-93. doi: 10.1007/BF02815234.

Abstract

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.

MeSH terms

  • Animals
  • Brain / metabolism*
  • Electron Transport Complex IV / metabolism*
  • Mitochondria / metabolism*
  • Oxidation-Reduction
  • Polarography
  • Proteins / isolation & purification
  • Proteins / metabolism*
  • Reference Values
  • Sheep
  • Swayback / metabolism*

Substances

  • Proteins
  • Electron Transport Complex IV