COMPROMISED FATTY-ACID OXIDATION IN MITOCHONDRIAL DISORDERS

Measurement of palmitate oxidation by the tritium release method in cu ltured fibroblasts or in lymphocytes detects patients with mitochondri al beta-oxidation disorders and in addition many patients with dysfunc tion of the respiratory chain. The clinical presentation and studies o f metabolite levels in serum and urine are valuable in the differentia l diagnosis between these two groups of disorders.