1-Deoxysphingolipids Encountered Exogenously and Made de Novo: Dangerous Mysteries inside an Enigma.
J Biol Chem
; 290(25): 15380-15389, 2015 Jun 19.
Article
in En
| MEDLINE
| ID: mdl-25947379
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.
Key words
Full text:
1
Database:
MEDLINE
Main subject:
Sphingosine
/
Peripheral Nervous System Diseases
/
Diabetes Mellitus
/
Serine C-Palmitoyltransferase
/
Non-alcoholic Fatty Liver Disease
/
Mutation
Limits:
Animals
/
Humans
Language:
En
Journal:
J Biol Chem
Year:
2015
Type:
Article