Oxysterols derived from cholesterol (WP4545)

The Oxysterol group of compounds are oxygenated derivatives of cholesterol or its sterol precursors, e.g. 7-dehydrocholesterol (7-DHC) or desmosterol. There are three mechanisms leading to the formation of oxysterols: 1. Enzymatically (first steps of sterol metabolism, being intermediates for the formation of steroid hormones, bile acids and 1,25-dihydroxyvitamin D3). 2. Non-enzymatically by encountering reactive oxygen species (ROS), providing a second pool of metabolites (this pool also includes oxidized cholesterol molecules taken in from diet), see [https://www.wikipathways.org/index.php/Pathway:WP5064 WP5064]. 3. Generation by the gut microflora and uptake through the enterohepatic circulation. Previously oxysterols where though to be inactive metabolic intermediates, however recent findings have established that these metabolites are involved in cholesterol homoeostasis, can be ligands to nuclear and G protein-coupled receptors and biomarkers of diseases (for example Niemann-Pick disease). This pathway drawing was inspired by Figure 3 of the review article by Griffiths et al. (2016) [https://www.ncbi.nlm.nih.gov/pubmed/27068984], and has been extended with immune system, receptor agonists, steroidal alkaloid and biomarker information from the same paper. This pathway has been updated with Figure 1 from Griffiths et al (2020) [https://dx.doi.org/10.1016%2Fj.prostaglandins.2019.106381] (green boxes), Figure 2 (yellow box) and Figure 3 (blue box).
last edited


DeSl, Conroy lipids, and Fehrhart

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Homo sapiens


Inborn Errors of Metabolism (IEM) Pathways Lipids and LIPID MAPS Rare Diseases


Disease Ontology: D-bifunctional protein deficiency Niemann-Pick disease type C1 alpha-methylacyl-CoA racemase deficiency congenital bile acid synthesis defect cerebrotendinous xanthomatosis congenital bile acid synthesis defect 6 Niemann-Pick disease type B Niemann-Pick disease Niemann-Pick disease type A

Pathway Ontology: cerebrotendinous xanthomatosis pathway classic metabolic pathway sterol regulatory element-binding protein signaling pathway cholesterol metabolic pathway familial hypercholanemia pathway


Label Type Compact Identifier
INSIG Protein uniprot:O15503
Cyp3A4 Protein uniprot:P08684
IL-17A Protein uniprot:Q16552
AKR1C4 GeneProduct hgnc.symbol:AKR1C4
Cyp7B1 Protein uniprot:O75881
7a,24R-Dihydroxy-3-oxocholest-4-en-(25R)26-oyl-CoA Metabolite lipidmaps:LMST04030256
BACS (SLC27A5) GeneProduct hgnc.symbol:SLC27A5
7α,12α−diHCO Metabolite lipidmaps:LMST04030114
CYP3A4 GeneProduct hgnc.symbol:CYP3A4
AKR1D1 GeneProduct hgnc.symbol:AKR1D1
7-beta, (25R)26-diHC Metabolite wikidata:Q27074030
7a-Hydroxy-3-oxocholest-4,24(E)-dien-26-oyl-CoA Metabolite lipidmaps:LMST04030258
CYP27A1 GeneProduct hgnc.symbol:CYP27A1
histamine Metabolite wikidata:Q61233
DHCR7 Protein uniprot:Q9UBM7
HSD3B7 Protein uniprot:Q9H2F3
ACOT1 Protein uniprot:Q86TX2
EBI2 Protein uniprot:P32249
7a-Hydroxy-3-oxochol-4-en-24-oyl-CoA Metabolite lipidmaps:LMST04010462
HSD3B7 GeneProduct hgnc.symbol:HSD3B7
CYP8B1 GeneProduct hgnc.symbol:CYP8B1
7α(25S)26-diHC Metabolite lipidmaps:LMST01010466
HSD3B7 Protein uniprot:Q9H2F3
DBP GeneProduct hgnc.symbol:DBP
LXR-alpha Protein uniprot:P13133
ACOX2 GeneProduct hgnc.symbol:ACOX2
3α,7α,12α−tri-H-5β-cholestan-(25R)26-oic acid Metabolite pubchem.compound:23657849
7-oxo-C and7beta-HC pathways Pathway wikipathways:WP5064
D8D7I Protein uniprot:Q15125
7a-Hydroxy-3-oxochol-4-en-24-oyl-taurine Metabolite lipidmaps:LMST05040022
DDA Metabolite wikidata:Q61983671
CYP27A1 GeneProduct hgnc.symbol:CYP27A1
BAAT GeneProduct hgnc.symbol:BAAT
EBI2 Protein uniprot:P32249
AMACR GeneProduct hgnc.symbol:AMACR
5β-CO-3α,7α,12α−(25R)26-tetrol Metabolite pubchem.compound:24771793
Cholesterol Metabolite wikidata:Q43656
7a,24S-Dihydroxy-3-oxocholest-4-en-(25R)26-oic acid Metabolite lipidmaps:LMST04030250
SCPx (SCP2) GeneProduct hgnc.symbol:SCP2
7α,25-diHCO Metabolite chebi:81013
AKR1C4 GeneProduct hgnc.symbol:AKR1C4
CYP3A4 GeneProduct hgnc.symbol:CYP3A4
Estrogen receptor alpha Protein uniprot:P03372
7a-Hydroxy-3-oxocholest-4-en-(25S)26-oyl-CoA Metabolite lipidmaps:LMST04030253
24S-HC Metabolite wikidata:Q27071873
7a-Hydroxy-3-oxocholest-4-en-(25R)26-oyl-CoA Metabolite lipidmaps:LMST04030252
7a,24S-Dihydroxy-3-oxocholest-4-en-(25R)26-oyl CoA Metabolite lipidmaps:LMST04030251
7α,12α,(25R)26-TriH-5β-CO Metabolite lipidmaps:LMST04030246
AKR1D1 GeneProduct hgnc.symbol:AKR1D1
LBP GeneProduct hgnc.symbol:LBP
CYP8B1 GeneProduct hgnc.symbol:CYP8B1
3β,7α-diHCA Metabolite wikidata:Q27154974
PXR Protein uniprot:O75469
7α,25-diHC Metabolite chebi:37623
7a-Hydroxy-3-oxochol-4-en-24-oic acid Metabolite inchikey:CFLVYJJIZHNITM-NLXMLWGDSA-N
7a-Hydroxy-3-oxochol-4-en-24-oyl-glycine Metabolite lipidmaps:LMST05030020
ROR-γt Protein uniprot:F1D8P6
7α−HCO Metabolite ChEBI
7a-Hydroxy-3,24-bisoxocholest-4-en-(25R)26-oyl-CoA Metabolite LIPID MAPS
ACOT Protein Enzyme Nomenclature
VLCS (SLC27A2) GeneProduct HGNC
5,6-alpha-Epoxycholesterol Metabolite Wikidata
Cholestane-3-beta,5-alpha, 6-beta-triol Metabolite Wikidata
7-beta-HC Metabolite Wikidata
7-alpha-HC Metabolite Wikidata
25-HC Metabolite Wikidata
7-alpha,25-diHC Metabolite LIPID MAPS
(25R)26-HC Metabolite Wikidata
7α,26-diHC Metabolite Wikidata
Cyp27A1 Protein Uniprot-TrEMBL
Cyp27A1 Protein Uniprot-TrEMBL
Cyp7A1 Protein Uniprot-TrEMBL
Cyp27A1 Protein Uniprot-TrEMBL
Cyp7B1 Protein Uniprot-TrEMBL
CH25H Protein Uniprot-TrEMBL
Cyp46A1 Protein Uniprot-TrEMBL
ChEH Protein Uniprot-TrEMBL
IL-17B Protein Uniprot-TrEMBL
IL-17C Protein Uniprot-TrEMBL
IL-17D Protein Uniprot-TrEMBL
IL-17E Protein Uniprot-TrEMBL
IL-17F Protein Uniprot-TrEMBL
Estrogen receptor beta Protein Uniprot-TrEMBL
LXR-beta Protein Uniprot-TrEMBL
7α−HCO Metabolite ChEBI
7-alpha-HC Metabolite Wikidata
7α(25S)26-diHCO Metabolite LIPID MAPS
7α,12α−diH-5β-CO Metabolite PubChem-compound
5β-CO-3α,7α,12α−triol Metabolite PubChem-compound
Cyp27A1 Protein Uniprot-TrEMBL
3β-HCA Metabolite PubChem-compound
Cyp27A1 Protein uniprot:Q02318
7α(25R)26−diHCO Metabolite pubchem.compound:11144200
7α-H-3-oxoC-4-en-(25R)26-oic acid Metabolite lipidmaps:LMST04030242
CYP27A1 GeneProduct hgnc.symbol:CYP27A1
CYP27A1 GeneProduct hgnc.symbol:CYP27A1
7α, 12α,(25R)26−triHCO Metabolite lipidmaps:LMST01010464
7α,12α,-H-3-oxoC-4-en-(25R)26-oic acid Metabolite inchikey:PEIQVFBLXUEBGA-GJKGPBNHSA-N
CYP8B1 GeneProduct hgnc.symbol:CYP8B1
7α,12α-diH-3-oxo-5β-CO-(25R)26-oic acid Metabolite inchikey:IPDDUDDXZPWYCG-RSIKEQKEQTSA-N
AKR1D1 GeneProduct hgnc.symbol:AKR1D1
CYP27A1 GeneProduct hgnc.symbol:CYP27A1
CYP8B1 GeneProduct hgnc.symbol:CYP8B1
7a,12a,25-TriH-cholest-4-en-3-one Metabolite pubchem.compound:5284265
7a,25-DiH-cholest-4-en-3-one Metabolite inchikey:POUKDTOWHPHYQU-HENOKILYSA-N
7a,25-DiH-3-oxocholest-4-en-26-oic acid Metabolite inchikey:DUYGXKURNABVMD-KOGONGFSSA-N
7a,12a-DiH-3-oxochol-4-en-24-oic acid Metabolite lipidmaps:LMST04010241
7a,12a,25-TriH-cholest-4-en-3,24-dione Metabolite lipidmaps:LMST04030249
Cholic acid Metabolite lipidmaps:LMST04010001
7a,12a,24,25-TetraH-cholest-4-en-3-one Metabolite lipidmaps:LMST04030248
HSD3B7 GeneProduct hgnc.symbol:HSD3B7
CYP39A1 GeneProduct hgnc.symbol:CYP39A1
7a,24S-diH,3O-CA Metabolite lipidmaps:LMST04030250
CYP27A1 GeneProduct hgnc.symbol:CYP27A1
AKR1C4 GeneProduct hgnc.symbol:AKR1C4
LXR-alpha Protein uniprot:P13133
LXR-beta Protein uniprot:P55055
24S-HC Metabolite wikidata:Q27071873
EBI2 Protein uniprot:P32249
7α-H-3-oxoC-4-en-(25R)26-oic acid Metabolite lipidmaps:LMST04030242
ACOT2 Protein uniprot:P49753
ACOT4 Protein uniprot:Q8N9L9
ACOT6 Protein uniprot:Q3I5F7
ACOT7 Protein uniprot:O00154
ACOT8 Protein uniprot:O14734
ACOT9 Protein uniprot:Q9Y305
ACOT11 Protein uniprot:Q8WXI4
ACOT12 Protein uniprot:Q8WYK0
ACOT13 Protein uniprot:Q9NPJ3
ACOT7L Protein uniprot:Q6ZUV0
ACOT15 Protein uniprot:Q8N1Q8
BACS (SLC27A5) GeneProduct hgnc.symbol:SLC27A5
VLCS (SLC27A2) GeneProduct hgnc.symbol:SLC27A2


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