Department of Biological Chemistry, Weizmann Institute of Science, Rehovot 76100, Israel.
Adv Exp Med Biol. 2010;688:60-71. doi: 10.1007/978-1-4419-6741-1_4.
Ceramide synthases (CerS) are integral membrane proteins of the endoplasmic reticulum. Six mammalian CerS have been described, with each utilizing fatty acyl CoAs of relatively defined chain lengths for N-acylation of the sphingoid long chain base. In this chapter, we review the main functional features of the CerS proteins, discuss their fatty acid specificity, kinetics, tissue distribution and mode of inhibition, as well as possible posttranslational modifications. We then address the reason that mammals contain six distinct CerS, whereas most other enzymes in the sphingolipid biosynthetic pathway only occur in one or two isoforms. Finally, we discuss the putative roles of CerS and the ceramide derived from the CerS, in signaling pathways and in development of disease.
神经酰胺合酶（CerS）是内质网的整合膜蛋白。已经描述了六种哺乳动物的 CerS，它们各自利用相对定义的链长的脂肪酸酰基辅酶 A 对神经鞘氨醇长链碱基进行 N-酰化。在本章中，我们回顾了 CerS 蛋白的主要功能特征，讨论了它们的脂肪酸特异性、动力学、组织分布和抑制模式，以及可能的翻译后修饰。然后，我们讨论了哺乳动物含有六种不同的 CerS 的原因，而鞘脂生物合成途径中的大多数其他酶只存在一种或两种同工酶。最后，我们讨论了 CerS 和 CerS 衍生的神经酰胺在信号通路和疾病发展中的可能作用。
