Fatty Acid Oxidation in Mitochondria from Needle Biopsy Samples of Human Skeletal Muscle

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Abstract
Activities for the oxidation of palmitoylcarnitine, of palmitoyl-CoA and of carnitine palmitoyltransferase were measured in mitochondria prepared from needle biopsy samples of human skeletal muscle. Results are presented for 9 normal subjects and 18 patients in whom there was evidence of mitochondrial abnormality. Palmitoylcarnitine and palmitoyl-CoA oxidation were measured spectrophotometrically by following the reduction of added cytochrome c in the presence of cyanide. Because of large variations in the activities between subjects it was essential to express the 3 activities per unit of cytochrome c oxidase activity to demonstrate unambiguous specific alterations in the activities. In most of the patients, the order of the 3 activities was similar to that in the normal subjects. In 5 cases the activity for palmitoylcarnitine oxidation was < 4% of the mean normal value. In 2 of these patients, the low activity could be accounted for by very low (< 10% normal) activity of carnitine palmitoyltransferase (CPT). In another 2 patients the activity of CPT was normal but that of palmitoyl-CoA dehydrogenase (a measure of .beta.-oxidation) was very low.

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