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 ceroid-lipofuscinosis, 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. (C) 1995 Wiley-Liss, Inc.