7-oxo-C and 7-beta-HC pathways (WP5064)
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); see [https://www.wikipathways.org/index.php/Pathway:WP4545 WP4545]. 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); described in this pathway. 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 describes Figure 4 and 5 from Griffiths et al (2020) [https://dx.doi.org/10.1016%2Fj.prostaglandins.2019.106381] and will be extended with disease information.
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Authors

Denise Slenter , Eric Weitz , and Egon Willighagen

Cited In

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Organism

Homo sapiens

Communities

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

Annotations

Pathway Ontology: cholesterol metabolic pathway classic metabolic pathway Smith-Lemli-Opitz Syndrome pathway

Disease Ontology: Niemann-Pick disease type A Niemann-Pick disease type B Niemann-Pick disease type C1 Smith-Lemli-Opitz syndrome Niemann-Pick disease type C2

Participants

Label Type Compact Identifier
7-Dehydrocholesterol Metabolite inchikey:UCTLRSWJYQTBFZ-DDPQNLDTSA-N
3b,5a,6b-Trihydroxycholan-24-oyl-glycine Metabolite inchikey:PVXZQUUYXFMXMF-ABSIFXOISA-N
3b,7b-Dihydroxychol-5-en-24-oic acid Metabolite inchikey:PXHCARRJGFGPAC-SZQOYVLDSA-N
Cholesterol Metabolite inchikey:HVYWMOMLDIMFJA-DPAQBDIFSA-N
7b-Hydroxycholesterol Metabolite inchikey:OYXZMSRRJOYLLO-KGZHIOMZSA-N
(25R)26-Hydroxy-7-oxocholesterol Metabolite inchikey:LFNAJBFFWWMSEW-HNFKANRHSA-N
Dendrogenin A Metabolite inchikey:AVFNYTPENXWWCA-BULFVYHESA-N
Cholestane-3b,5a,6b,(25R)26-tetrol Metabolite inchikey:PFYSRSDOSXYIFG-JKYVJSSTSA-N
3b,5a,6b-Trihydroxycholestan-(25R)26-oic acid Metabolite inchikey:LMWUOMGULHFSQR-HDEGCWFHSA-N
3b-Hydroxy-7-oxochol-5-en-24-oyl-CoA Metabolite inchikey:HEPNPBUPTRNGJT-ILFWFKRZSA-N
Cholestane-3b,5a,6b-triol Metabolite inchikey:YMMFNKXZULYSOQ-RUXQDQFYSA-N
7-Oxocholesterol Metabolite inchikey:YIKKMWSQVKJCOP-ABXCMAEBSA-N
3b,7b-Dihydroxychol-5-en-24-oyl-glycine Metabolite inchikey:BMRAURDLHOPSBE-FRMRTHFDSA-N
7b-Peroxycholesterol Metabolite inchikey:KJIGLXGIVLBXCF-UOQFGJKXSA-N
3b-Hydroxy-7-oxocholest-5-en-(25R)26-oic acid Metabolite inchikey:QOEPZHFZXUROGV-BXDHRDAUSA-N
3b-Hydroxy-7-oxochol-5-en-24-oic acid Metabolite inchikey:JHFXTNJNQOZGEJ-HICUSVRDSA-N
3b,5a,6b-Trihydroxycholan-24-oic acid Metabolite inchikey:NMKAZCXSXYNCFW-DWQVTILUSA-N
3b-Hydroxy-7-oxochol-5-en-24-oyl-glycine Metabolite inchikey:AXBXXYALPXTOOI-UBKHJBOVSA-N
3b,5a-DiH-cholestan-6-one Metabolite inchikey:SJZZRXMQSAXCFD-FRAMIZMOSA-N
3b,7b-Dihydroxycholest-5-en-(25R)26-oic acid Metabolite inchikey:GYJSAWZGYQXRBS-WMYDBBFWSA-N
3b,5a,6b-Trihydroxycholan-24-oyl-CoA Metabolite inchikey:WRWBXZATSFAUGF-UJKOPXFNSA-N
3b,7b-Dihydroxychol-5-en-24-oyl-CoA Metabolite inchikey:XRRIYAPQOCXHCP-NRNJIOKESA-N
3b,24R-Dihydroxy-7-oxocholest-5-en-(25R)26-oyl-CoA Metabolite inchikey:UOHZUNSDJHLARD-JLLVOJIKSA-N
3b,5a,6b,24R-Tetrahydroxycholestan-(25R)26-oyl-CoA Metabolite inchikey:PJJHUKDTGITXML-MFOUGJSISA-N
5,6-Epoxycholesterol Metabolite inchikey:PRYIJAGAEJZDBO-XXGHXXDPSA-N
7b,(25R)26-Dihydroxycholesterol Metabolite inchikey:RXMHNAKZMGJANZ-BMOLSTJGSA-N
3b,7b,24R-Trihydroxycholest-5-en-(25R)26-oyl-CoA Metabolite inchikey:BZALPCSTIFZGTP-YBYDULIXSA-N
Cholesterol Metabolite inchikey:HVYWMOMLDIMFJA-DPAQBDIFSA-N
Cholesterol Metabolite inchikey:HVYWMOMLDIMFJA-DPAQBDIFSA-N
CYP7A1 GeneProduct hgnc.symbol:CYP7A1
NPC1 GeneProduct hgnc.symbol:NPC1
HSD11B1 GeneProduct hgnc.symbol:HSD11B1
HSD11B2 GeneProduct hgnc.symbol:HSD11B2
CYP27A1 GeneProduct hgnc.symbol:CYP27A1
BACS (SLC27A5) GeneProduct hgnc.symbol:SLC27A5
VLCS (SLC27A2) GeneProduct hgnc.symbol:SLC27A2
AMACR GeneProduct hgnc.symbol:AMACR
ACOX2 GeneProduct hgnc.symbol:ACOX2
DBP GeneProduct hgnc.symbol:DBP
SCPx (SCP2) GeneProduct hgnc.symbol:SCP2
DBP GeneProduct hgnc.symbol:DBP
BAAT GeneProduct hgnc.symbol:BAAT
CYP27A1 GeneProduct hgnc.symbol:CYP27A1
BACS (SLC27A5) GeneProduct hgnc.symbol:SLC27A5
VLCS (SLC27A2) GeneProduct hgnc.symbol:SLC27A2
AMACR GeneProduct hgnc.symbol:AMACR
ACOX2 GeneProduct hgnc.symbol:ACOX2
DBP GeneProduct hgnc.symbol:DBP
SCPx (SCP2) GeneProduct hgnc.symbol:SCP2
DBP GeneProduct hgnc.symbol:DBP
BAAT GeneProduct hgnc.symbol:BAAT
HSD11B2 GeneProduct hgnc.symbol:HSD11B2
HSD11B1 GeneProduct hgnc.symbol:HSD11B1
HSD11B2 GeneProduct hgnc.symbol:HSD11B2
HSD11B1 GeneProduct hgnc.symbol:HSD11B1
HSD11B2 GeneProduct hgnc.symbol:HSD11B2
NPC2 GeneProduct hgnc.symbol:NPC2
ACOT1 Protein uniprot:Q86TX2
ACOT Protein eccode:3.1.2.2
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
ChEH Protein uniprot:P34913
ACOT1 Protein uniprot:Q86TX2
ACOT Protein eccode:3.1.2.2
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
DHCR7 Protein uniprot:Q9UBM7
D8D7I Protein uniprot:Q15125
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References

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