Synthesis of ceramides and 1-deoxyceramides (WP5194)

Homo sapiens

Synthesis of ceramides and 1-deoxyceramides from serine and alanine, respectively, including the synthesis of complex glycosphingolipids in humans. Multiple more complex sphingolipid synthesis reactions were added to improve pathway versatility. 1-deoxyceramide synthesis shows a lot of similarities with ceramide synthesis but does not come close in terms of documentation, hence this comprehensive figure.


Quinten Lok and Denise Slenter


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Homo sapiens



Pathway Ontology

ceramide signaling pathway sphingolipid biosynthetic pathway lacto-series glycosphingolipid metabolic pathway

Cell Type Ontology

animal cell


Label Type Compact URI Comment
alanine Metabolite chebi:16449
ceramide Metabolite chebi:52639
sphingosine-1-phosphate Metabolite chebi:37550
3-ketosphinganine Metabolite chebi:17862
Hexadecenal Metabolite hmdb:HMDB0060482
serine Metabolite chebi:17822
Lc3Cer Metabolite hmdb:HMDB0062485
1-O-acyl-ceramide Metabolite chebi:133444
Phosphoethanolamine Metabolite chebi:17553
GA2 Metabolite chebi:36774
CerP(d18:1/16:0) Metabolite chebi:72963
ganglioside GM4 Metabolite chebi:27499
sulphatide Metabolite chebi:18318
Sphinganine Metabolite hmdb:HMDB0000269
Sphingosine Metabolite hmdb:HMDB0000252
Dihydroceramide Metabolite hmdb:HMDB0006752
Galactosylceramide Metabolite chebi:18390
lactosylceramide Metabolite chebi:134507
globoside Gb3Cer Metabolite chebi:88154
S1P Metabolite chebi:37550
sphingomyelins Metabolite chebi:62490
Palmitoyl-CoA Metabolite chebi:15525
Glucosylceramide Metabolite chebi:18368
isogloboside iGb3Cer Metabolite chebi:140817
1-Deoxyceramide Metabolite chebi:52639
1-Deoxy-3-ketosphinganine Metabolite chebi:17862
1-deoxysphinganine Metabolite chebi:67109
1-Deoxysphingosine Metabolite hmdb:HMDB0000252
1-Deoxydihydroceramide Metabolite hmdb:HMDB0006752
CERS4 GeneProduct ensembl:ENSG00000090661
UGT8 GeneProduct ensembl:ENSG00000174607
UGCG GeneProduct ensembl:ENSG00000148154
B3GNT5 GeneProduct ensembl:ENSG00000176597
KDSR GeneProduct ensembl:ENSG00000119537
DGAT1 GeneProduct ensembl:ENSG00000185000 Uncertain, source material did not specify what protein isoform is responsible for this reaction.
B4GALNT1 GeneProduct ensembl:ENSG00000135454
CERK GeneProduct ensembl:ENSG00000100422
ASAH1 GeneProduct ensembl:ENSG00000104763
A4GALT GeneProduct ensembl:ENSG00000128274
CERS1 GeneProduct ensembl:ENSG00000223802
A3GALT2 GeneProduct ensembl:ENSG00000184389
SGMS2 GeneProduct ensembl:ENSG00000164023
CERS2 GeneProduct ensembl:ENSG00000143418
SPHK1 GeneProduct ensembl:ENSG00000176170
SGMS1 GeneProduct ensembl:ENSG00000198964
CERS3 GeneProduct ensembl:ENSG00000154227
DES GeneProduct ensembl:ENSG00000175084
B4GALT1 GeneProduct ensembl:ENSG00000086062
CERS5 GeneProduct ensembl:ENSG00000139624
CERS6 GeneProduct ensembl:ENSG00000172292
CERS4 GeneProduct ensembl:ENSG00000090661
CERS1 GeneProduct ensembl:ENSG00000223802
CERS2 GeneProduct ensembl:ENSG00000143418
CERS3 GeneProduct ensembl:ENSG00000154227
CERS5 GeneProduct ensembl:ENSG00000139624
CERS6 GeneProduct ensembl:ENSG00000172292
ASAH1 GeneProduct ensembl:ENSG00000104763
SPTLC1 Protein uniprot:O15269


  1. Targeting Glucosylceramide Synthesis in the Treatment of Rare and Common Renal Disease. Shayman JA. Semin Nephrol. 2018 Mar;38(2):183–92. PubMed Europe PMC Scholia
  2. 1-Deoxyceramides - Key players in lipotoxicity and progression to type 2 diabetes? Sen P, Hyötyläinen T, Orešič M. Acta Physiol (Oxf). 2021 May;232(1):e13635. PubMed Europe PMC Scholia
  3. Convergence: Lactosylceramide-Centric Signaling Pathways Induce Inflammation, Oxidative Stress, and Other Phenotypic Outcomes. Chatterjee S, Balram A, Li W. Int J Mol Sci. 2021 Feb 12;22(4):1816. PubMed Europe PMC Scholia