Label Type Identifier Comment Ensembl NCBI gene HGNC UniProt Wikidata ChEBI InChI 7-Dehydrocholesterol Metabolite lipidmaps:LMST01010069 "aka 7-DHC
abundant in SLOS." wikidata:Q139350 chebi:17759;chebi:17759 inchikey:UCTLRSWJYQTBFZ-DDPQNLDTSA-N 3b,5a,6b-Trihydroxycholan-24-oyl-glycine Metabolite lipidmaps:LMST05030019 "" 3b,7b-Dihydroxychol-5-en-24-oic acid Metabolite lipidmaps:LMST04010218 "" Cholesterol Metabolite lipidmaps:LMST01010001 "" wikidata:Q43656 chebi:16113;chebi:16113 inchikey:HVYWMOMLDIMFJA-DPAQBDIFSA-N 7b-Hydroxycholesterol Metabolite lipidmaps:LMST01010047 "" wikidata:Q27074031 chebi:42989;chebi:42989 inchikey:OYXZMSRRJOYLLO-KGZHIOMZSA-N (25R)26-Hydroxy-7-oxocholesterol Metabolite lipidmaps:LMST04030180 "" wikidata:Q27159806 chebi:87653;chebi:87653 inchikey:LFNAJBFFWWMSEW-HNFKANRHSA-N Dendrogenin A Metabolite lipidmaps:LMST05050025 "" wikidata:Q61983671 inchikey:AVFNYTPENXWWCA-BULFVYHESA-N Cholestane-3b,5a,6b,(25R)26-tetrol Metabolite lipidmaps:LMST01010458 "" 3b,5a,6b-Trihydroxycholestan-(25R)26-oic acid Metabolite lipidmaps:LMST04030238 "" 3b-Hydroxy-7-oxochol-5-en-24-oyl-CoA Metabolite lipidmaps:LMST01010461 "" Cholestane-3b,5a,6b-triol Metabolite lipidmaps:LMST01010052 "" wikidata:Q27103493 chebi:28082;chebi:28082 inchikey:YMMFNKXZULYSOQ-RUXQDQFYSA-N 7-Oxocholesterol Metabolite lipidmaps:LMST01010049 "aka 7-OC
Elevated levels found in Wolman's disease" wikidata:Q27133201 chebi:64294;chebi:64294 inchikey:YIKKMWSQVKJCOP-ABXCMAEBSA-N 3b,7b-Dihydroxychol-5-en-24-oyl-glycine Metabolite lipidmaps:LMST05030017 "" 7b-Peroxycholesterol Metabolite lipidmaps:LMST01010459 "" wikidata:Q105141844 inchikey:KJIGLXGIVLBXCF-KGZHIOMZSA-N 3b-Hydroxy-7-oxocholest-5-en-(25R)26-oic acid Metabolite lipidmaps:LMST04030239 "" wikidata:Q27159930 chebi:87783;chebi:87783 inchikey:QOEPZHFZXUROGV-BXDHRDAUSA-N 3b-Hydroxy-7-oxochol-5-en-24-oic acid Metabolite lipidmaps:LMST04010394 "" wikidata:Q76294977 chebi:178721 inchikey:JHFXTNJNQOZGEJ-HICUSVRDSA-N 3b,5a,6b-Trihydroxycholan-24-oic acid Metabolite lipidmaps:LMST04010339 "" wikidata:Q76294832 chebi:187658 inchikey:NMKAZCXSXYNCFW-DWQVTILUSA-N 3b-Hydroxy-7-oxochol-5-en-24-oyl-glycine Metabolite lipidmaps:LMST05030018 "" 3b,5a-DiH-cholestan-6-one Metabolite lipidmaps:LMST01010126 "aka 3b,5a-Dihydroxycholestan-6-one" wikidata:Q27896404 chebi:166811;chebi:166811 inchikey:SJZZRXMQSAXCFD-ZCBMJONGSA-N 3b,7b-Dihydroxycholest-5-en-(25R)26-oic acid Metabolite lipidmaps:LMST04030235 "" 3b,5a,6b-Trihydroxycholan-24-oyl-CoA Metabolite lipidmaps:LMST04010459 "" 3b,7b-Dihydroxychol-5-en-24-oyl-CoA Metabolite lipidmaps:LMST04010460 "" 3b,24R-Dihydroxy-7-oxocholest-5-en-(25R)26-oyl-CoA Metabolite lipidmaps:LMST04030262 "" 3b,5a,6b,24R-Tetrahydroxycholestan-(25R)26-oyl-CoA Metabolite lipidmaps:LMST04030244 "" 5a,6a-Epoxycholesterol Metabolite lipidmaps:LMST01010011 "" wikidata:Q27121609 chebi:49305;chebi:49305 inchikey:PRYIJAGAEJZDBO-ZEQHCUNVSA-N 7b,(25R)26-Dihydroxycholesterol Metabolite lipidmaps:LMST04030178 "" wikidata:Q27074030 inchikey:RXMHNAKZMGJANZ-BMOLSTJGSA-N 3b,7b,24R-Trihydroxycholest-5-en-(25R)26-oyl-CoA Metabolite lipidmaps:LMST04030261 "" Cholesterol Metabolite lipidmaps:LMST01010001 "" wikidata:Q43656 chebi:16113;chebi:16113 inchikey:HVYWMOMLDIMFJA-DPAQBDIFSA-N Cholesterol Metabolite lipidmaps:LMST01010001 "" wikidata:Q43656 chebi:16113;chebi:16113 inchikey:HVYWMOMLDIMFJA-DPAQBDIFSA-N CYP7A1 GeneProduct hgnc.symbol:CYP7A1 "" ensembl:ENSG00000167910 ncbigene:1581 hgnc.symbol:CYP7A1 uniprot:P22680 NPC1 GeneProduct hgnc.symbol:NPC1 "" ensembl:ENSG00000141458 ncbigene:4864 hgnc.symbol:NPC1 uniprot:K7ERW2;uniprot:K7ERM4;uniprot:O15118;uniprot:K7EIH7;uniprot:K7ENZ0;uniprot:K7EQ23 HSD11B1 GeneProduct hgnc.symbol:HSD11B1 "" ensembl:ENSG00000117594 ncbigene:3290 hgnc.symbol:HSD11B1 uniprot:P28845;uniprot:X5D2L1;uniprot:A0A087WU76;uniprot:A0A0A0MQV1 HSD11B2 GeneProduct hgnc.symbol:HSD11B2 "" ensembl:ENSG00000176387 ncbigene:3291 hgnc.symbol:HSD11B2 uniprot:R4GN04;uniprot:P80365 CYP27A1 GeneProduct hgnc.symbol:CYP27A1 "" ensembl:ENSG00000135929 ncbigene:1593 hgnc.symbol:CYP27A1 uniprot:Q02318;uniprot:F8WD90;uniprot:C9J1K5 BACS (SLC27A5) GeneProduct hgnc.symbol:SLC27A5 "Bile Acid CoA ligase (or synthetase)
microsomal protein mostly expressed in liver [PMID:24309898, 25409824(mouse)]" ensembl:ENSG00000083807 ncbigene:10998 hgnc.symbol:SLC27A5 uniprot:Q9Y2P5;uniprot:M0R075 VLCS (SLC27A2) GeneProduct hgnc.symbol:SLC27A2 "very-long chain acyl-CoA synthetase
expressed mostly in liver and kidney, and present in ER and peroxisome [PMID:24309898, 25409824(mouse)]" ensembl:ENSG00000140284 ncbigene:11001 hgnc.symbol:SLC27A2 uniprot:O14975;uniprot:G3V1R7 AMACR GeneProduct hgnc.symbol:AMACR "alpha-methylacyl-CoA racemase
broadly expressed [PMID:24309898, 25409824(mouse)]" ensembl:ENSG00000242110 ncbigene:23600 hgnc.symbol:AMACR uniprot:D6RB81;uniprot:Q9UHK6 ACOX2 GeneProduct hgnc.symbol:ACOX2 "" ensembl:ENSG00000168306 ncbigene:8309 hgnc.symbol:ACOX2 uniprot:C9JY29;uniprot:H7C4Q2;uniprot:C9J0G0;uniprot:H7C573;uniprot:Q99424;uniprot:H7C4Y2 DBP GeneProduct hgnc.symbol:DBP "D-biofinctional protein; aka MFE2, HSD17B4" ensembl:ENSG00000105516 ncbigene:1628 hgnc.symbol:DBP uniprot:Q10586;uniprot:M0QXW3;uniprot:M0QXP1 SCPx (SCP2) GeneProduct hgnc.symbol:SCP2 "" ensembl:ENSG00000116171 ncbigene:6342 hgnc.symbol:SCP2 uniprot:A0A384NY87;uniprot:H0YCB0;uniprot:H0YF61;uniprot:H0YD06;uniprot:E9PLD1;uniprot:P22307;uniprot:H0YEU8 DBP GeneProduct hgnc.symbol:DBP "D-biofinctional protein; aka MFE2, HSD17B4" ensembl:ENSG00000105516 ncbigene:1628 hgnc.symbol:DBP uniprot:Q10586;uniprot:M0QXW3;uniprot:M0QXP1 BAAT GeneProduct ncbigene:570 "amino acid N-acyl transferase" ensembl:ENSG00000136881 ncbigene:570 hgnc.symbol:BAAT uniprot:A0A6Q8PF60;uniprot:A0A6Q8PHR9;uniprot:Q14032 CYP27A1 GeneProduct hgnc.symbol:CYP27A1 "" ensembl:ENSG00000135929 ncbigene:1593 hgnc.symbol:CYP27A1 uniprot:Q02318;uniprot:F8WD90;uniprot:C9J1K5 BACS (SLC27A5) GeneProduct hgnc.symbol:SLC27A5 "Bile Acid CoA ligase (or synthetase)
microsomal protein mostly expressed in liver [PMID:24309898, 25409824(mouse)]" ensembl:ENSG00000083807 ncbigene:10998 hgnc.symbol:SLC27A5 uniprot:Q9Y2P5;uniprot:M0R075 VLCS (SLC27A2) GeneProduct hgnc.symbol:SLC27A2 "very-long chain acyl-CoA synthetase
expressed mostly in liver and kidney, and present in ER and peroxisome [PMID:24309898, 25409824(mouse)]" ensembl:ENSG00000140284 ncbigene:11001 hgnc.symbol:SLC27A2 uniprot:O14975;uniprot:G3V1R7 AMACR GeneProduct hgnc.symbol:AMACR "alpha-methylacyl-CoA racemase
broadly expressed [PMID:24309898, 25409824(mouse)]" ensembl:ENSG00000242110 ncbigene:23600 hgnc.symbol:AMACR uniprot:D6RB81;uniprot:Q9UHK6 ACOX2 GeneProduct hgnc.symbol:ACOX2 "" ensembl:ENSG00000168306 ncbigene:8309 hgnc.symbol:ACOX2 uniprot:C9JY29;uniprot:H7C4Q2;uniprot:C9J0G0;uniprot:H7C573;uniprot:Q99424;uniprot:H7C4Y2 DBP GeneProduct hgnc.symbol:DBP "D-biofinctional protein; aka MFE2, HSD17B4" ensembl:ENSG00000105516 ncbigene:1628 hgnc.symbol:DBP uniprot:Q10586;uniprot:M0QXW3;uniprot:M0QXP1 SCPx (SCP2) GeneProduct hgnc.symbol:SCP2 "" ensembl:ENSG00000116171 ncbigene:6342 hgnc.symbol:SCP2 uniprot:A0A384NY87;uniprot:H0YCB0;uniprot:H0YF61;uniprot:H0YD06;uniprot:E9PLD1;uniprot:P22307;uniprot:H0YEU8 DBP GeneProduct hgnc.symbol:DBP "D-biofinctional protein; aka MFE2, HSD17B4" ensembl:ENSG00000105516 ncbigene:1628 hgnc.symbol:DBP uniprot:Q10586;uniprot:M0QXW3;uniprot:M0QXP1 BAAT GeneProduct hgnc.symbol:BAAT "amino acid N-acyl transferase" ensembl:ENSG00000136881 ncbigene:570 hgnc.symbol:BAAT uniprot:A0A6Q8PF60;uniprot:A0A6Q8PHR9;uniprot:Q14032 HSD11B2 GeneProduct hgnc.symbol:HSD11B2 "" ensembl:ENSG00000176387 ncbigene:3291 hgnc.symbol:HSD11B2 uniprot:R4GN04;uniprot:P80365 HSD11B1 GeneProduct hgnc.symbol:HSD11B1 "" ensembl:ENSG00000117594 ncbigene:3290 hgnc.symbol:HSD11B1 uniprot:P28845;uniprot:X5D2L1;uniprot:A0A087WU76;uniprot:A0A0A0MQV1 HSD11B2 GeneProduct hgnc.symbol:HSD11B2 "" ensembl:ENSG00000176387 ncbigene:3291 hgnc.symbol:HSD11B2 uniprot:R4GN04;uniprot:P80365 HSD11B1 GeneProduct hgnc.symbol:HSD11B1 "" ensembl:ENSG00000117594 ncbigene:3290 hgnc.symbol:HSD11B1 uniprot:P28845;uniprot:X5D2L1;uniprot:A0A087WU76;uniprot:A0A0A0MQV1 HSD11B2 GeneProduct hgnc.symbol:HSD11B2 "" ensembl:ENSG00000176387 ncbigene:3291 hgnc.symbol:HSD11B2 uniprot:R4GN04;uniprot:P80365 NPC2 GeneProduct hgnc.symbol:NPC2 "" ensembl:ENSG00000119655 ncbigene:10577 hgnc.symbol:NPC2 uniprot:E7EMS2;uniprot:H0YIZ1;uniprot:G3V3D1;uniprot:P61916;uniprot:A0A024R6C0;uniprot:G3V2V8;uniprot:H0YJE2;uniprot:G3V3E8;uniprot:J3KMY5 ACOT1 Protein uniprot:Q86TX2 "" ensembl:ENSG00000184227 ncbigene:641371 hgnc.symbol:ACOT1 uniprot:G3V4F2;uniprot:Q86TX2;uniprot:E9KL42 ACOT Protein eccode:3.1.2.2 "acyl-CoA thioesterase are a group of enzymes" ACOT2 Protein uniprot:P49753 "'originally thought to be in peroxisome [PMID:10944470)], later found to be mitochondrial [PMID:16940157]' [https://www.uniprot.org/uniprot/P49753]" ensembl:ENSG00000119673 ncbigene:10965 hgnc.symbol:ACOT2 uniprot:P49753;uniprot:F6VI00;uniprot:A0A087WT95 ACOT4 Protein uniprot:Q8N9L9 "'Compared to mouse peroxisomal succinyl-coenzyme A thioesterase/ACOT4, the human enzyme has a broad substrate specificity overlapping the activity of three mouse acyl-coenzyme A thioesterases, providing an explanation for the unexpectedly low number of acyl-coenzyme A thioesterase genes in the human genome [PMID:16940157]' [https://www.uniprot.org/uniprot/Q8N9L9]" ensembl:ENSG00000177465 ncbigene:122970 hgnc.symbol:ACOT4 uniprot:Q8N9L9 ACOT6 Protein uniprot:Q3I5F7 "" ensembl:ENSG00000205669 ncbigene:641372 hgnc.symbol:ACOT6 uniprot:Q3I5F7;uniprot:G3V3W6 ACOT7 Protein uniprot:O00154 "" ensembl:ENSG00000097021 ncbigene:11332 hgnc.symbol:ACOT7 uniprot:K7EKP8;uniprot:O00154 ACOT8 Protein uniprot:O14734 "" ensembl:ENSG00000101473 ncbigene:10005 hgnc.symbol:ACOT8 uniprot:H7C5A7;uniprot:E9PJN0;uniprot:F6VBM3;uniprot:E9PIS4;uniprot:Q9BR14;uniprot:O14734;uniprot:A0A494C0N6;uniprot:H0Y698;uniprot:E9PRD4 ACOT9 Protein uniprot:Q9Y305 "" ensembl:ENSG00000123130 ncbigene:23597 hgnc.symbol:ACOT9 uniprot:Q9Y305;uniprot:C9J7L8;uniprot:F8WDI2;uniprot:H7C5Q2 ACOT11 Protein uniprot:Q8WXI4 "" ensembl:ENSG00000162390 ncbigene:26027 hgnc.symbol:ACOT11 uniprot:Q8WXI4 ACOT12 Protein uniprot:Q8WYK0 "" ensembl:ENSG00000172497 ncbigene:134526 hgnc.symbol:ACOT12 uniprot:Q8WYK0 ACOT13 Protein uniprot:Q9NPJ3 "" ensembl:ENSG00000112304 ncbigene:55856 hgnc.symbol:ACOT13 uniprot:Q9NPJ3 ACOT7L Protein uniprot:Q6ZUV0 "'Could be the product of a pseudogene. The peptide used to produce antibodies against ACOT7L matches at 85% with ACOT7 and the antibodies may not be specific to ACOT7L.' [https://www.uniprot.org/uniprot/Q6ZUV0]" ACOT15 Protein uniprot:Q8N1Q8 "" ensembl:ENSG00000196407 ncbigene:284486 hgnc.symbol:THEM5 uniprot:Q8N1Q8;uniprot:H0Y6P4 ChEH Protein uniprot:P34913 "AKA cholesterol epoxide hydrolase (ChEH); EC: 3.3.2.11
'ChEH is a dimer of 7-dehydrocholesterol reductase (DHCR7) and 3β-hydroxysteroid-Δ8-Δ7-isomerase (D8D7I)'" ensembl:ENSG00000120915 ncbigene:2053 hgnc.symbol:EPHX2 uniprot:E5RFU2;uniprot:H0YAW7;uniprot:E5RFH6;uniprot:P34913;uniprot:E5RI53 ACOT1 Protein uniprot:Q86TX2 "" ensembl:ENSG00000184227 ncbigene:641371 hgnc.symbol:ACOT1 uniprot:G3V4F2;uniprot:Q86TX2;uniprot:E9KL42 ACOT Protein eccode:3.1.2.2 "acyl-CoA thioesterase are a group of enzymes" ACOT2 Protein uniprot:P49753 "'originally thought to be in peroxisome [PMID:10944470)], later found to be mitochondrial [PMID:16940157]' [https://www.uniprot.org/uniprot/P49753]" ensembl:ENSG00000119673 ncbigene:10965 hgnc.symbol:ACOT2 uniprot:P49753;uniprot:F6VI00;uniprot:A0A087WT95 ACOT4 Protein uniprot:Q8N9L9 "'Compared to mouse peroxisomal succinyl-coenzyme A thioesterase/ACOT4, the human enzyme has a broad substrate specificity overlapping the activity of three mouse acyl-coenzyme A thioesterases, providing an explanation for the unexpectedly low number of acyl-coenzyme A thioesterase genes in the human genome [PMID:16940157]' [https://www.uniprot.org/uniprot/Q8N9L9]" ensembl:ENSG00000177465 ncbigene:122970 hgnc.symbol:ACOT4 uniprot:Q8N9L9 ACOT6 Protein uniprot:Q3I5F7 "" ensembl:ENSG00000205669 ncbigene:641372 hgnc.symbol:ACOT6 uniprot:Q3I5F7;uniprot:G3V3W6 ACOT7 Protein uniprot:O00154 "" ensembl:ENSG00000097021 ncbigene:11332 hgnc.symbol:ACOT7 uniprot:K7EKP8;uniprot:O00154 ACOT8 Protein uniprot:O14734 "" ensembl:ENSG00000101473 ncbigene:10005 hgnc.symbol:ACOT8 uniprot:H7C5A7;uniprot:E9PJN0;uniprot:F6VBM3;uniprot:E9PIS4;uniprot:Q9BR14;uniprot:O14734;uniprot:A0A494C0N6;uniprot:H0Y698;uniprot:E9PRD4 ACOT9 Protein uniprot:Q9Y305 "" ensembl:ENSG00000123130 ncbigene:23597 hgnc.symbol:ACOT9 uniprot:Q9Y305;uniprot:C9J7L8;uniprot:F8WDI2;uniprot:H7C5Q2 ACOT11 Protein uniprot:Q8WXI4 "" ensembl:ENSG00000162390 ncbigene:26027 hgnc.symbol:ACOT11 uniprot:Q8WXI4 ACOT12 Protein uniprot:Q8WYK0 "" ensembl:ENSG00000172497 ncbigene:134526 hgnc.symbol:ACOT12 uniprot:Q8WYK0 ACOT13 Protein uniprot:Q9NPJ3 "" ensembl:ENSG00000112304 ncbigene:55856 hgnc.symbol:ACOT13 uniprot:Q9NPJ3 ACOT7L Protein uniprot:Q6ZUV0 "'Could be the product of a pseudogene. The peptide used to produce antibodies against ACOT7L matches at 85% with ACOT7 and the antibodies may not be specific to ACOT7L.' [https://www.uniprot.org/uniprot/Q6ZUV0]" ACOT15 Protein uniprot:Q8N1Q8 "" ensembl:ENSG00000196407 ncbigene:284486 hgnc.symbol:THEM5 uniprot:Q8N1Q8;uniprot:H0Y6P4 DHCR7 Protein uniprot:Q9UBM7 "'ChEH is a dimer of 7-dehydrocholesterol reductase (DHCR7) and 3β-hydroxysteroid-Δ8-Δ7-isomerase (D8D7I)'
the 3beta-hydroxysteroid delta7 reductase (DHCR7), which is the regulatory subunit. [https://en.wikipedia.org/wiki/Cholesterol-5,6-oxide_hydrolase]" ensembl:ENSG00000172893 ncbigene:1717 hgnc.symbol:DHCR7 uniprot:A0A804HI25;uniprot:A0A804HL56;uniprot:E9PQ71;uniprot:E9PJ54;uniprot:B4E1K5;uniprot:H0YCS7;uniprot:H0YE57;uniprot:Q9UBM7;uniprot:E9PM00;uniprot:A0A024R5F7;uniprot:A0A804HJQ7;uniprot:E9PIP9;uniprot:H0YEJ5;uniprot:A0A804HJI8;uniprot:E9PRL8;uniprot:E9PLZ2 D8D7I Protein uniprot:Q15125 "'ChEH is a dimer of 7-dehydrocholesterol reductase (DHCR7) and 3β-hydroxysteroid-Δ8-Δ7-isomerase (D8D7I)'
also known as the emopamyl binding protein (EBP), which is the catalytic subunit [https://en.wikipedia.org/wiki/Cholesterol-5,6-oxide_hydrolase]" ensembl:ENSG00000147155 ncbigene:10682 hgnc.symbol:EBP uniprot:A0A024QYX0;uniprot:C9J719;uniprot:Q15125;uniprot:C9JJ78