Ceramidase and ceramide synthesis in human kidney and cerebellum. Description of a new alkaline ceramidase.
It has been shown that tissues of patients with Farber's disease characteristically lack acid (pH 4.0) ceramidase. In normal cerebellum, however, ceramide cleavage and the reverse reaction, free fatty acid-dependent ceramide synthesis, both occur not only at pH 4.0 but also at pH 9.0, although normal kidney exhibits these activities only at pH 4.0. Both tissues are capable of snythesizing ceramide via an acyl-COA-dependent pathway at neutral pH. The synthetic analog of ceramide, N-oleoyl-ethanolamine, is a potent inhibitor of ceramidase.
D000581:Amidohydrolases / Q000378:metabolism*; D002518:Ceramides / Q000096:biosynthesis*; D002531:Cerebellum / Q000378:metabolism*; D006801:Humans; D006863:Hydrogen-Ion Concentration; D007231:Infant, Newborn; D007592:Joint Diseases / Q000151:congenital / Q000201:enzymology; D007668:Kidney / Q000378:metabolism*; D007700:Kinetics; D008064:Lipidoses / Q000151:congenital / Q000201:enzymology; D012127:Respiratory Distress Syndrome, Newborn / Q000201:enzymology; D013577:Syndrome
D002518:Ceramides; D000581:Amidohydrolases