Biosynthesis and turnover of 1-deoxy-sphingoid bases (WP5179)
Homo sapiens
Biosynthesis and turnover of 1-deoxy-sphingoid bases (1-doexySLs). 1-Deoxysphingolipids differ structurally from canonical SLs as they lack the essential C1-OH group. Consequently, 1-deoxysphingolipids cannot be converted to complex sphingolipids and are not degraded over the canonical catabolic pathways. Further conversion of 1-deoxysphingosine is done by members of the CYP4A/F family. It is however not clear which specific genes are involved in these processes.
Authors
Roel Van Overbeek , Denise Slenter , and Eric WeitzActivity
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Organisms
Homo sapiensCommunities
Annotations
Disease Ontology
hereditary sensory and autonomic neuropathy type 1 diabetes mellitusPathway Ontology
classic metabolic pathway sphingolipid biosynthetic pathway sphingolipid metabolic pathway sphingolipidosis pathway| Label | Type | Compact URI | Comment |
|---|---|---|---|
| 1-Deoxysphinganine-2OH | Metabolite | inchikey:AADNOUNRPCOGMI-BFWZDYSYSA-N | InChiKey based on converting structural drawing from Carreira [31132366] Fig. 5, to SMILES. |
| 1-Deoxysphingosine | Metabolite | cas:193222-34-3 | InChI-Key which might be relevant (since CAS number does not provide linkouts): BPUUVXWJPCOCMG-ZWKOTPCHSA-N |
| 1-Deoxyceramide | Metabolite | lipidmaps:LMSP0206 | |
| 1-Deoxysphingosine-OH | Metabolite | inchikey:KSKFOMCZXQEJBA-PXKIYYGHSA-N | InChiKey based on converting structural drawing from Carreira [31132366] Fig. 5, to SMILES. |
| 1-Deoxysphinganine-OH | Metabolite | inchikey:BJWFMCNWESUWNO-PXKIYYGHSA-N | InChiKey based on converting structural drawing from Carreira [31132366] Fig. 5, to SMILES. |
| 1-Deoxysphingadiene | Metabolite | inchikey:KSKFOMCZXQEJBA-PXKIYYGHSA-N | InChiKey based on converting structural drawing from Carreira [31132366] Fig. 5, to SMILES. |
| 1-Deoxy-3-ketosphinganine | Metabolite | chebi:67176 | |
| Palmitoyl-CoA | Metabolite | chebi:15525 | |
| CoA (22:0) | Metabolite | lipidmaps:LMFA07050289 | |
| CoA (24:0) | Metabolite | lipidmaps:LMFA07050372 | |
| L-alanine | Metabolite | chebi:16977 | |
| CoA (16:0) | Metabolite | lipidmaps:LMFA07050360 | |
| CoA (18:0) | Metabolite | lipidmaps:LMFA07050369 | |
| 1-Deoxysphinganine | Metabolite | chebi:67106 | |
| ATP | Metabolite | chebi:30616 | |
| H2O | Metabolite | chebi:15377 | |
| CoA (24:1) | Metabolite | lipidmaps:LMFA07050098 | |
| 1-Deoxy-dihydroceramide | Metabolite | chebi:67111 | |
| ADP | Metabolite | chebi:456216 | |
| CoA (20:0) | Metabolite | chebi:15527 | |
| CoA (26:0) | Metabolite | lipidmaps:LMFA07050327 | |
| CYP4A | GeneProduct | eccode:1.14.14.80 | |
| CYP4F | GeneProduct | eccode:1.14.14.94 | |
| KDSR | GeneProduct | ensembl:ENSG00000119537 | |
| SGPP1 | GeneProduct | ensembl:ENSG00000126821 | |
| ASAH1 | GeneProduct | ensembl:ENSG00000104763 | |
| SPTLC2 | GeneProduct | ensembl:ENSG00000100596 | |
| SPHK2 | GeneProduct | ensembl:ENSG00000063176 | |
| SPTLC1 | GeneProduct | ensembl:ENSG00000090054 | |
| SPHK1 | GeneProduct | ensembl:ENSG00000176170 | |
| SGPP2 | GeneProduct | ensembl:ENSG00000163082 |
References
- The cytochrome P450 4 (CYP4) family. Simpson AE. Gen Pharmacol. 1997 Mar;28(3):351–9. PubMed Europe PMC Scholia
- The revised human liver cytochrome P450 “Pie”: absolute protein quantification of CYP4F and CYP3A enzymes using targeted quantitative proteomics. Michaels S, Wang MZ. Drug Metab Dispos. 2014 Aug;42(8):1241–51. PubMed Europe PMC Scholia
- 1-Deoxysphingolipids Encountered Exogenously and Made de Novo: Dangerous Mysteries inside an Enigma. Duan J, Merrill AH Jr. J Biol Chem. 2015 Jun 19;290(25):15380–9. PubMed Europe PMC Scholia
- 1-Deoxysphingolipids. Lone MA, Santos T, Alecu I, Silva LC, Hornemann T. Biochim Biophys Acta Mol Cell Biol Lipids. 2019 Apr;1864(4):512–21. PubMed Europe PMC Scholia
- Quantifying 1-deoxydihydroceramides and 1-deoxyceramides in mouse nervous system tissue. Schwartz NU, Mileva I, Gurevich M, Snider J, Hannun YA, Obeid LM. Prostaglandins Other Lipid Mediat. 2019 Apr;141:40–8. PubMed Europe PMC Scholia
- Mammalian sphingoid bases: Biophysical, physiological and pathological properties. Carreira AC, Santos TC, Lone MA, Zupančič E, Lloyd-Evans E, de Almeida RFM, et al. Prog Lipid Res. 2019 Jul;75:100988. PubMed Europe PMC Scholia
- Metabolism of HSAN1- and T2DM-associated 1-deoxy-sphingolipids inhibits the migration of fibroblasts. Karsai G, Steiner R, Kaech A, Lone MA, von Eckardstein A, Hornemann T. J Lipid Res. 2021;62:100122. PubMed Europe PMC Scholia