Palmitate oxidation in human muscle: comparison to CPT and carnitine.
The evaluating of palmitate oxidation in muscle tissue may be a useful screening test for detecting defects in fatty acid metabolism in human neuromuscular disease. If the test is to be useful, it is necessary to obtain data on a wide variety of muscle illnesses for comparative purposes. We report our experience with palmitate oxidation, muscle carnitine, and carnitine palmityl transferase (CPT) activity in 148 muscles biopsies from a variety of illnesses. The efficacy of using total protein, citrate synthase, and (1-14C) pyruvate oxidation as internal references was investigated. Palmitate oxidation was significantly less than normal (P less than or equal to 0.01) in Duchenne muscular dystrophy, congenital nonprogressive myopathy, congenital muscular dystrophy, malignant hyperpyrexia, and denervation, depending on the internal reference used. Muscle carnitine levels followed a similar pattern, however, CPT activity did not. The possibility of these findings being secondary to inactivity is discussed.
D000217:Acyltransferases / Q000378:metabolism*; D000293:Adolescent; D000328:Adult; D000368:Aged; D002331:Carnitine / Q000032:analysis / Q000378:metabolism*; D002334:Carnitine O-Palmitoyltransferase / Q000032:analysis / Q000378:metabolism*; D002648:Child; D002675:Child, Preschool; D006801:Humans; D007223:Infant; D008875:Middle Aged; D009132:Muscles / Q000032:analysis / Q000378:metabolism*; D009468:Neuromuscular Diseases / Q000378:metabolism; D010168:Palmitates / Q000032:analysis / Q000378:metabolism*; D010169:Palmitic Acids / Q000378:metabolism*
D010168:Palmitates; D010169:Palmitic Acids; D000217:Acyltransferases; D002334:Carnitine O-Palmitoyltransferase; D002331:Carnitine