Erythrocyte membrane reacylation in juvenile neuronal ceroid-lipofuscinosis: Measurement of membrane-bound carnitine palmitoyl transferase, acyl-CoA synthetase, and lysophospholipid: Acyl-CoA acyltransferase activities

In order to study the biochemical mechanisms responsible for the membrane fatty acid deficiency in juvenile neuronal ceroidlipofuscinosis, we have analyzed the reacylation pathway in isolated erythrocyte membranes in 5 patients. We studied membrane carnitine palmitoyl transferase, and developed a combined assay to study acyl-CoA synthetase and lysophospholipid acyl-CoA acyltransferase activities. There were no significant differences between control and patient membranes, suggesting that abnormalities in these 3 putative candidate enzymes are not responsible for the disease.