1-Deoxysphingolipids Encountered Exogenously and Made de Novo: Dangerous Mysteries inside an Enigma.
1-Deoxysphingolipids Encountered Exogenously and Made de Novo: Dangerous Mysteries inside an Enigma.
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DOI：
10.1074/jbc.r115.658823
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发表时间：
2015-06-19
期刊：
影响因子：
--
通讯作者：
Merrill AH Jr
中科院分区：
文献类型：
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作者：
Duan J;Merrill AH Jr
哺乳动物鞘脂的传统骨架是由丝氨酸棕榈酰转移酶（SPT）产生的2-氨基，1，3-二醇。许多生物体还产生非传统的、细胞毒性的1-脱氧鞘氨醇碱，并且令人惊讶的是，哺乳动物SPT也生物合成其中的一些（例如从l-丙氨酸合成1-脱氧鞘氨醇）。这些被迅速N-酰化为1-脱氧-“神经酰胺”，具有非常罕见的生物物理性质。1-脱氧鞘脂的功能尚不清楚，但当通过遗传性SPT突变升高时，它们肯定是危险的感觉和自主神经病变的贡献者，并且它们在糖尿病，非酒精性脂肪性肝炎，丝氨酸缺乏症和其他疾病中是明显的。这些物质既是食物的组成部分，又是内源性的，是谜中之谜。
The traditional backbones of mammalian sphingolipids are 2-amino, 1,3-diols made by serine palmitoyltransferase (SPT). Many organisms additionally produce non-traditional, cytotoxic 1-deoxysphingoid bases and, surprisingly, mammalian SPT biosynthesizes some of them, too (e.g. 1-deoxysphinganine from l-alanine). These are rapidly N-acylated to 1-deoxy-“ceramides” with very uncommon biophysical properties. The functions of 1-deoxysphingolipids are not known, but they are certainly dangerous as contributors to sensory and autonomic neuropathies when elevated by inherited SPT mutations, and they are noticeable in diabetes, non-alcoholic steatohepatitis, serine deficiencies, and other diseases. As components of food as well as endogenously produced, these substances are mysteries within an enigma.