AbstractMitochondria from the cerebral cortex of untreated lambs from areas in which enzootic ataxia occurred showed a lower content of haem a (from cytochromes a and a 3) than mitochondria from control lambs with adequate copper but the haems of cytochromcs b2 c1 and c were not depleted. Cytochrome oxidase activity was closely correlated with the content of haem a (P<0.001) but there was no correlation with total mitochondrial copper which was always present in molar exccss of haem a. It is concluded that the lowered cytochrome oxidase activity in mitochondria from untreated lambs is immediately attributable to depletion of haem a. In clinically ataxic lambs that showed degradation of myelin in thc spinal cord, brain cytochrome oxidase was depressed by no more t han 60%. Arguments are advanced that this depletion was not sufficiently severe to have led to respiratory constraint