鞘糖脂的新陈代谢与生理学功能（英文）

鞘糖脂由一个神经酰胺的脂骨架与一个或多个糖基连接形成,存在于细胞膜中,承担多种生理学功能。我们将对鞘糖脂的生物化学及生理学方面研究做一概述,随后简要介绍近年来鞘糖脂的临床研究进展。在讨论鞘糖脂生物化学方面的研究中,我们把重点放在介绍鞘脂类及鞘糖脂的结构和生合成途径。脂类生物合成和降解是通过一系列酶的参与紧密调控的,如果一种酶参与代谢失败会导致酶底物的大量堆积,会引起溶酶体贮积症,这种疾病是由具有分解代谢活性的水解酶缺失所造成的。随后,我们介绍鞘糖脂在细胞及动物体内的生理学方面的功能,以及鞘糖脂在临床方面的一些病症中所起的作用,即使许多细节还有待于进一步研究。

The Metabolism and functional roles of glycosphingolipids

Glycosphingolipids （GSLs）, comprising a ceramide lipid backbone linked to one/more saccharides, are contained in a various cell membranes and have recently been implicated in many physiologic functions. We now present an overview of GSLs bio- chemistry and physiology, followed by a brief presentation of recent advances in clinical research related to GSLs. In discussing GSL bio- chemistry, we focus on the structure of sphingolipids and GSL, and their biosynthetic pathways. Biosynthesis and subsequently degrada- tion of these lipids is tightly regulated via the. involvement of numerous enzymes, and failure of an enzyme to participate in the metabolism results in storage of the enzyme＇s substrate, giving rise to a lysosomal storage disease, caused by a defect in the catabolic ac- tivity of a hydrolase. We then discuss some physiological function of GSLs in cell physiology in vitro and in vivo. We end with a discus- sion of GSL in the clinical aspects of a number of diseases, even though many of the details remain to be elucidated.