title: 1-Deoxysphingolipids Encountered Exogenously and Made de Novo: Dangerous Mysteries inside an Enigma.
abstract: 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.
authors.affiliation: Schools of Biology and Chemistry & Biochemistry, and the Parker H. Petit Institute for Bioengineering and Bioscience, Georgia Institute of Technology, Atlanta, Georgia 30332.
authors.forename: Jingjing
authors.initials: J
authors.lastname: Duan
authors.affiliation: Schools of Biology and Chemistry & Biochemistry, and the Parker H. Petit Institute for Bioengineering and Bioscience, Georgia Institute of Technology, Atlanta, Georgia 30332. Electronic address: al.merrill@biology.gatech.edu.
authors.forename: Alfred H
authors.initials: AH
authors.lastname: Merrill
mesh_terms: D000409:Alanine / Q000235:genetics / Q000378:metabolism; D000818:Animals; D003920:Diabetes Mellitus / Q000235:genetics / Q000378:metabolism / Q000473:pathology; D006801:Humans; D008055:Lipids; D009154:Mutation; D065626:Non-alcoholic Fatty Liver Disease / Q000235:genetics / Q000378:metabolism / Q000473:pathology; D010523:Peripheral Nervous System Diseases / Q000235:genetics / Q000378:metabolism / Q000473:pathology; D051102:Serine C-Palmitoyltransferase / Q000235:genetics / Q000378:metabolism; D013110:Sphingosine / Q000031:analogs & derivatives* / Q000235:genetics / Q000378:metabolism / Q000633:toxicity
chemical_list: D008055:Lipids; D051102:Serine C-Palmitoyltransferase; D013110:Sphingosine; D000409:Alanine; C408363:spisulosine
keywords: 1-deoxysphingolipid; alanine; cancer; ceramide; diabetes; fumonisin; neuropathy; serine; serine palmitoyltransferase; sphingolipid