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.


Roel Van Overbeek and Denise Slenter


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Homo sapiens



Pathway Ontology

sphingolipidosis pathway sphingolipid biosynthetic pathway sphingolipid metabolic pathway classic metabolic pathway

Disease Ontology

hereditary sensory and autonomic neuropathy type 1 diabetes mellitus


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:
CYP4F GeneProduct eccode:
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


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