Sugita M, Willians M, Dulaney J T, Moser H W
Biochim Biophys Acta. 1975 Jul 22;398(1):125-31. doi: 10.1016/0005-2760(75)90176-9.
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.
已表明，法伯病患者的组织特征性地缺乏酸性（pH 4.0）神经酰胺酶。然而，在正常小脑中，神经酰胺的裂解以及逆向反应，即游离脂肪酸依赖性神经酰胺合成，不仅在pH 4.0时发生，在pH 9.0时也会发生，尽管正常肾脏仅在pH 4.0时表现出这些活性。两种组织都能够在中性pH下通过酰基辅酶A依赖性途径合成神经酰胺。神经酰胺的合成类似物N-油酰乙醇胺是神经酰胺酶的有效抑制剂。
