期刊
JOURNAL OF BIOLOGICAL CHEMISTRY
卷 290, 期 25, 页码 15380-15389出版社
AMER SOC BIOCHEMISTRY MOLECULAR BIOLOGY INC
DOI: 10.1074/jbc.R115.658823
关键词
cancer; ceramide; diabetes; serine; serine palmitoyltransferase; sphingolipid; 1-deoxysphingolipid; alanine; neuropathy; fumonisin
资金
- National Institutes of Health [GM076217]
- Smithgall Institute Endowed Chair in Molecular Cell Biology at Georgia Tech
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.
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