Ceramidase and ceramide synthesis in human kidney and cerebellum: description of a new alkaline ceramidase

M Sugita, M Williams, JT Dulaney, HW Moser - Biochimica et Biophysica …, 1975 - Elsevier
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 synthesizing ceramide via an acyl-CoA-dependent pathway at neutral pH. The
synthetic analog of ceramide, N-oleoyl-ethanolamine, is a potent inhibitor of ceramidase.