Biosynthesis and turnover of 1-deoxy-sphingoid bases (WP5179)

Homo sapiens

Open in new tab
Download PNG Download SVG Download JSON Download GPML Learn more about Downloads
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 and Denise Slenter

Activity

last edited

Discuss this pathway

Check for ongoing discussions or start your own.

Cited In

Are you planning to include this pathway in your next publication? See How to Cite and add a link here to your paper once it's online.

Organisms

Homo sapiens

Communities

Annotations

Pathway Ontology

sphingolipidosis pathway sphingolipid biosynthetic pathway sphingolipid metabolic pathway classic metabolic pathway

Disease Ontology

hereditary sensory and autonomic neuropathy type 1 diabetes mellitus

Participants
Query Drugst.One

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

  1. The cytochrome P450 4 (CYP4) family. Simpson AE. Gen Pharmacol. 1997 Mar;28(3):351–9. PubMed Europe PMC Scholia
  2. 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
  3. 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
  4. 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
  5. 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
  6. 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
  7. 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