Park Joo-Won, Pewzner-Jung Yael
Department of Biochemistry, School of Medicine, Ewha Womans University, Seoul 158-710, South Korea.
Handb Exp Pharmacol. 2013(215):89-107. doi: 10.1007/978-3-7091-1368-4_5.
The ceramide synthase (CerS) enzymes catalyze the formation of (dihydro) ceramide, and thereby provide critical complexity to all sphingolipids (SLs) with respect to their acyl chain length. This review summarizes the progress in the field of CerS from the time of their discovery more than a decade ago as Longevity assurance (Lass) genes in yeast, until the recent development of CerS-deficient mouse models. Human hereditary CerS disorders are yet to be discovered. However, the recent findings in CerS mutant animals highlight the important physiological role of these enzymes. The fundamental findings with respect to CerS structure, function, localization, and regulation are discussed, as well as CerS roles in maintaining longevity in vivo.
神经酰胺合酶(CerS)催化(二氢)神经酰胺的形成,从而在所有鞘脂(SLs)的酰基链长度方面提供关键的复杂性。本综述总结了从十多年前在酵母中作为长寿保障(Lass)基因被发现以来,CerS领域的进展,直至CerS缺陷小鼠模型的最新发展。人类遗传性CerS疾病尚未被发现。然而,最近在CerS突变动物中的发现突出了这些酶的重要生理作用。本文讨论了关于CerS结构、功能、定位和调节的基本发现,以及CerS在体内维持长寿中的作用。