Bile acid and bile salt metabolism (Homo sapiens)

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23, 33, 36, 39994, 154, 15116, 14, 1628, 37, 446, 14, 1624, 34, 451325, 4026, 274448334, 7, 1532, 493314, 7, 15915, 2033335, 2013226, 14, 169153310, 17, 3814, 156, 14, 16224, 154, 7, 1524, 34, 45134, 1519, 314, 156, 14, 1632, 496, 14, 1612230333324, 7, 158, 21, 4335, 4112, 4247333, 29, 306, 14, 16333324, 34, 45183335, 4113336, 14, 16191461633333344335, 20925, 4024, 34, 451825, 404, 154, 15bile salts and acids complexed with albumin mitochondrial matrixalbumincholate bile salt complex peroxisomal matrixbile salts and acids bile salts and acids HSD17B4 dimer bile salts and acids complexed with FABP6 endoplasmic reticulum lumenendoplasmic reticulum lumenmitochondrial matrixACOX2FAD endoplasmic reticulum lumenendoplasmic reticulum lumencytosolcytosolendoplasmic reticulum lumenalbuminbile salt and acid endoplasmic reticulum lumenbile salts and acids albuminbile salt complex cytosolCH25H bile salts cytosolcholate bile salts NADPHH+5beta-cholestan-3alpha,7alpha,24CYP27A1H+NADP+Cytochrome P450 4-cholesten-7alpha-ol-3-one5beta-cholestan-3alpha,7alpha,12alpha-triolNADHAldo-keto reductase familyNADPHH2OPiSLC10A225Aldo-keto reductase familyTHCA5beta-cholestan-3alpha,7alpha,12alpha,24chenodeoxycholoyl-CoAcholate 4-cholesten-7alpha, 12alpha-diol-3-one4-cholesten-7alpha,27-diol-3-onePPiH+O25beta-cholestan-7alpha,12alpha,27-triol-3-oneH+Na+NADP+ADPNADPHH2ONADHO2bile salts and acids ALB O27 alpha-hydroxycholesterolO2NADP+O2taurocholate O2TetraHCA5beta-cholestan-7alpha,24ALBHSD3B7ALB taurochenodeoxycholate bile salts and acids complexed with albuminALB H+CoA-SHcholate NAD+5beta-cholestan-3alpha, 7alpha, 12alpha-triolglycochenodeoxycholate ATPH+SLCO1A224-hydroxycholesterolcholateH2Oglycocholate Fe2+ H2ODHCAO2SLCO1B3Cholest-5-ene-3beta,7alpha,24CYP27A1FABP63alpha,7alpha-dihydroxy-5beta-cholestan-26-alNADPHNADP+NAD+NADPHNADP+25Cholest-5-ene-3beta,7alpha,25-trioltaurochenodeoxycholate CholesterolATPSLCO1B1glycine; taurine25NADPH5beta-cholestan-3alpha,7alpha,12alpha,24albumincholate bile salt complexAMACRNADPH3alpha,7alpha,24HSD3B7glycocholate cholate; chenodeoxycholate5beta-cholestan-3alpha,7alpha,26-triolH2O25CoA-SHO2ATPNADPHNADP+NADHH2O25H2OATPPPiCholesterol3alpha,7alpha,12alpha-trihydroxy-5beta-cholest-24-one-CoAAMPbile salts5beta-cholesten-7alpha, 12alpha-diol-3-onecholoyl-CoA; chenodeoxycholoyl-CoASLC27A5H2Oalbuminbile salt and acid albuminbile salt complexSLC27A5Cholesterolglycocholate taurocholate bile saltsH+H+NADP+ACOX2 PPi3alpha,7alpha,12alpha-trihydroxy-5beta-cholestan-27-alH+24-hydroxycholesterolCoA-SH27-hydroxycholesterolFABP6 H2OFAD taurochenodeoxycholate H+25NADPHNa+5beta-cholestan-3alpha,7alpha,12alpha,27-tetrolH2OTHCABAATSLC27A5NADPHH2Oglycine; taurineCytochrome P450 25-hydroxycholesterolCoA-SHNADP+NADPHO2H+H+ATPNa+3,7,24THCA-CoA5beta-cholestan-3alpha,7alpha,26-triolH+Cytochrome P450 NAD+HSD17B4CH25H 4-cholesten-7alpha,12alpha,24ADPHSD17B4 dimerHSD3B7bile saltsCoA-SHchenodeoxycholate 5beta-cholestan-3alpha,7alpha,12alpha,27-tetrolH+NADPHAMP5beta-cholestan-3alpha,7alpha,24NADPHPiO2TetraHCASLC10A1taurocholate O224-hydroxycholesterolCytochrome P450 NADP+H2ONADPHH+O2propionyl CoANADPHglycochenodeoxycholate NADP+cholateH+NADP+Cholest-5-ene-3beta,7alpha,27-triolAKR1D125SLC27A2chenodeoxycholate NADP+27-hydroxycholesterolO2CYP27A1AMPH+CHOLbile salts and acids complexed with FABP6NADPHNADHSLC27A2H+25CYP27A1chenodeoxycholate bile saltsNADPH3,7,24THCANADP+NADPHCoA-SHH+Cytochrome P450 ABCB11Na+glycochenodeoxycholate H+H2ONADP+5beta-cholestan-3alpha,7alpha,245beta-cholestan-3alpha, 7alpha-diolCYP27A13alpha,7alpha-dihydroxy-5beta-cholest-24-one-CoAH+NADP+ACOX2FADCoA-SHNADP+bile salts and acidsO2H2OABCC3SCP2-15beta-cholestan-3alpha,7alpha,12alpha,24NADP+H+H2OCytochrome P450 H2OH+NADP+4-cholesten-7alpha,24H+H2OH+3alpha,7alpha,12alpha,24NADP+NADP+NAD+taurocholate 27HCHOLH+CYP39A1CYP46A1NADPHTHCA-CoAALB bile saltsH2O5beta-cholestan-3alpha,7alpha-diolcholate bile saltsAldo-keto reductase familyH2OBAATO2H2O25beta-cholestan-7alpha,12alpha,244-cholesten-7alpha,12alpha,27-triol-3-oneAKR1D1AKR1D15beta-cholestan-7alpha-ol-3-oneCYP27A1chenodeoxycholate NADP+NADPH5beta-cholestan-7alpha,27-diol-3-oneglycocholate NADPH3,7,24THCAcholate bile saltsH2ONADPHtaurocholate H2Oglycocholate CH25H DHCAcholoyl-CoACYP27A1ACOT8cholate NAD+NADP+O2253,7,24THCA-CoAtaurochenodeoxycholate


Description

In a healthy adult human, about 500 mg of cholesterol is converted to bile salts daily. Newly synthesized bile salts are secreted into the bile and released into the small intestine where they emulsify dietary fats (Russell 2003). About 95% of the bile salts in the intestine are recovered and returned to the liver (Kullak-Ublick et al. 2004; Trauner and Boyer 2002). The major pathway for bile salt synthesis in the liver begins with the conversion of cholesterol to 7alpha-hydroxycholesterol. Bile salt synthesis can also begin with the synthesis of an oxysterol - 24-hydroxycholesterol or 27-hydroxycholesterol. In the body, the initial steps of these two pathways occur in extrahepatic tissues, generating intermediates that are transported to the liver and converted to bile salts via the 7alpha-hydroxycholesterol pathway. These extrahepatic pathways contribute little to the total synthesis of bile salts, but are thought to play important roles in extrahepatic cholesterol homeostasis (Javitt 2002). Original Pathway at Reactome: http://www.reactome.org/PathwayBrowser/#DB=gk_current&FOCUS_SPECIES_ID=48887&FOCUS_PATHWAY_ID=194068

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Bibliography

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History

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CompareRevisionActionTimeUserComment
114948view16:47, 25 January 2021ReactomeTeamReactome version 75
113392view11:46, 2 November 2020ReactomeTeamReactome version 74
112597view15:57, 9 October 2020ReactomeTeamReactome version 73
104892view15:14, 23 June 2019EgonwReplaced a secondary ChEBI identifiers with a primary identifier.
101513view11:37, 1 November 2018ReactomeTeamreactome version 66
101049view21:19, 31 October 2018ReactomeTeamreactome version 65
100580view19:53, 31 October 2018ReactomeTeamreactome version 64
100129view16:38, 31 October 2018ReactomeTeamreactome version 63
99679view15:08, 31 October 2018ReactomeTeamreactome version 62 (2nd attempt)
99272view12:45, 31 October 2018ReactomeTeamreactome version 62
93944view13:46, 16 August 2017ReactomeTeamreactome version 61
93534view11:26, 9 August 2017ReactomeTeamreactome version 61
86633view09:22, 11 July 2016ReactomeTeamreactome version 56
83139view10:08, 18 November 2015ReactomeTeamVersion54
81480view13:01, 21 August 2015ReactomeTeamVersion53
76958view08:24, 17 July 2014ReactomeTeamFixed remaining interactions
76663view12:03, 16 July 2014ReactomeTeamFixed remaining interactions
75992view10:05, 11 June 2014ReactomeTeamRe-fixing comment source
75695view11:03, 10 June 2014ReactomeTeamReactome 48 Update
75051view13:56, 8 May 2014AnweshaFixing comment source for displaying WikiPathways description
74695view08:46, 30 April 2014ReactomeTeamReactome46
44976view14:24, 6 October 2011MartijnVanIerselOntology Term : 'bile acid biosynthetic pathway' added !
42156view23:17, 4 March 2011MaintBotModified categories
42011view21:50, 4 March 2011MaintBotAutomatic update
39814view05:51, 21 January 2011MaintBotNew pathway

External references

DataNodes

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NameTypeDatabase referenceComment
24-hydroxycholesterolMetaboliteCHEBI:34310 (ChEBI)
25-hydroxycholesterolMetaboliteCHEBI:42977 (ChEBI)
25MetaboliteCHEBI:15494 (ChEBI)
25MetaboliteCHEBI:27393 (ChEBI)
25MetaboliteCHEBI:27458 (ChEBI)
25MetaboliteCHEBI:37642 (ChEBI)
25MetaboliteCHEBI:37643 (ChEBI)
27-hydroxycholesterolMetaboliteCHEBI:17703 (ChEBI)
27HCHOLMetaboliteCHEBI:17703 (ChEBI)
3,7,24THCA-CoAMetaboliteCHEBI:27403 (ChEBI)
3,7,24THCAMetaboliteCHEBI:27403 (ChEBI)
3alpha,7alpha,12alpha,24MetaboliteCHEBI:63849 (ChEBI)
3alpha,7alpha,12alpha-trihydroxy-5beta-cholest-24-one-CoAMetaboliteCHEBI:27379 (ChEBI)
3alpha,7alpha,12alpha-trihydroxy-5beta-cholestan-27-alMetaboliteCHEBI:16466 (ChEBI)
3alpha,7alpha,24MetaboliteCHEBI:63848 (ChEBI)
3alpha,7alpha-dihydroxy-5beta-cholest-24-one-CoAMetaboliteCHEBI:28533 (ChEBI)
3alpha,7alpha-dihydroxy-5beta-cholestan-26-alMetaboliteCHEBI:27428 (ChEBI)
4-cholesten-7alpha, 12alpha-diol-3-oneMetaboliteCHEBI:28477 (ChEBI)
4-cholesten-7alpha,12alpha,24MetaboliteCHEBI:63839 (ChEBI)
4-cholesten-7alpha,12alpha,27-triol-3-oneMetaboliteCHEBI:48333 (ChEBI)
4-cholesten-7alpha,24MetaboliteCHEBI:63838 (ChEBI)
4-cholesten-7alpha,27-diol-3-oneMetaboliteCHEBI:48825 (ChEBI)
4-cholesten-7alpha-ol-3-oneMetaboliteCHEBI:17899 (ChEBI)
5beta-cholestan-3alpha, 7alpha, 12alpha-triolMetaboliteCHEBI:16496 (ChEBI)
5beta-cholestan-3alpha, 7alpha-diolMetaboliteCHEBI:28047 (ChEBI)
5beta-cholestan-3alpha,7alpha,12alpha,24MetaboliteCHEBI:17278 (ChEBI)
5beta-cholestan-3alpha,7alpha,12alpha,24MetaboliteCHEBI:63834 (ChEBI)
5beta-cholestan-3alpha,7alpha,12alpha,24MetaboliteCHEBI:63835 (ChEBI)
5beta-cholestan-3alpha,7alpha,12alpha,27-tetrolMetaboliteCHEBI:17278 (ChEBI)
5beta-cholestan-3alpha,7alpha,12alpha-triolMetaboliteCHEBI:16496 (ChEBI)
5beta-cholestan-3alpha,7alpha,24MetaboliteCHEBI:63831 (ChEBI)
5beta-cholestan-3alpha,7alpha,24MetaboliteCHEBI:63833 (ChEBI)
5beta-cholestan-3alpha,7alpha,26-triolMetaboliteCHEBI:28540 (ChEBI)
5beta-cholestan-3alpha,7alpha-diolMetaboliteCHEBI:28047 (ChEBI)
5beta-cholestan-7alpha,12alpha,24MetaboliteCHEBI:63830 (ChEBI)
5beta-cholestan-7alpha,12alpha,27-triol-3-oneMetaboliteCHEBI:48834 (ChEBI)
5beta-cholestan-7alpha,24MetaboliteCHEBI:63829 (ChEBI)
5beta-cholestan-7alpha,27-diol-3-oneMetaboliteCHEBI:48778 (ChEBI)
5beta-cholestan-7alpha-ol-3-oneMetaboliteCHEBI:2290 (ChEBI)
5beta-cholesten-7alpha, 12alpha-diol-3-oneMetaboliteCHEBI:2288 (ChEBI)
7 alpha-hydroxycholesterolMetaboliteCHEBI:17500 (ChEBI)
ABCB11ProteinO95342 (Uniprot-TrEMBL)
ABCC3ProteinO15438 (Uniprot-TrEMBL)
ACOT8ProteinO14734 (Uniprot-TrEMBL)
ACOX2 FADComplexREACT_10603 (Reactome)
ACOX2 ProteinQ99424 (Uniprot-TrEMBL)
ADPMetaboliteCHEBI:16761 (ChEBI)
AKR1D1ProteinP51857 (Uniprot-TrEMBL)
ALB ProteinP02768 (Uniprot-TrEMBL)
ALBProteinP02768 (Uniprot-TrEMBL)
AMACRProteinQ9UHK6 (Uniprot-TrEMBL)
AMPMetaboliteCHEBI:16027 (ChEBI)
ATPMetaboliteCHEBI:15422 (ChEBI)
Aldo-keto reductase familyProteinREACT_164934 (Reactome) This CandidateSet contains sequences identified by William Pearson's analysis of Reactome catalyst entities. Catalyst entity sequences were used to identify analagous sequences that shared overall homology and active site homology. Sequences in this Candidate set were identified in an April 24, 2012 analysis.
BAATProteinQ14032 (Uniprot-TrEMBL)
CH25H ProteinO95992 (Uniprot-TrEMBL)
CH25H ComplexREACT_10164 (Reactome)
MetaboliteCHEBI:27403 (ChEBI)
MetaboliteCHEBI:52050 (ChEBI)
CHOLMetaboliteCHEBI:16113 (ChEBI)
CYP27A1ProteinQ02318 (Uniprot-TrEMBL)
CYP39A1ProteinQ9NYL5 (Uniprot-TrEMBL)
CYP46A1ProteinQ9Y6A2 (Uniprot-TrEMBL)
Cholest-5-ene-3beta,7alpha,24MetaboliteCHEBI:37640 (ChEBI)
Cholest-5-ene-3beta,7alpha,25-triolMetaboliteCHEBI:37623 (ChEBI)
Cholest-5-ene-3beta,7alpha,27-triolMetaboliteCHEBI:18431 (ChEBI)
CholesterolMetaboliteCHEBI:16113 (ChEBI)
CoA-SHMetaboliteCHEBI:15346 (ChEBI)
Cytochrome P450 ProteinREACT_160403 (Reactome) This CandidateSet contains sequences identified by William Pearson's analysis of Reactome catalyst entities. Catalyst entity sequences were used to identify analagous sequences that shared overall homology and active site homology. Sequences in this Candidate set were identified in an April 24, 2012 analysis.
Cytochrome P450 ProteinREACT_164336 (Reactome) This CandidateSet contains sequences identified by William Pearson's analysis of Reactome catalyst entities. Catalyst entity sequences were used to identify analagous sequences that shared overall homology and active site homology. Sequences in this Candidate set were identified in an April 24, 2012 analysis.
Cytochrome P450 ProteinREACT_165338 (Reactome) This CandidateSet contains sequences identified by William Pearson's analysis of Reactome catalyst entities. Catalyst entity sequences were used to identify analagous sequences that shared overall homology and active site homology. Sequences in this Candidate set were identified in an April 24, 2012 analysis.
DHCAMetaboliteCHEBI:16577 (ChEBI)
FABP6 ProteinP51161 (Uniprot-TrEMBL)
FABP6ProteinP51161 (Uniprot-TrEMBL)
FAD MetaboliteCHEBI:16238 (ChEBI)
Fe2+ MetaboliteCHEBI:18248 (ChEBI)
H+MetaboliteCHEBI:15378 (ChEBI)
H2O2MetaboliteCHEBI:16240 (ChEBI)
H2OMetaboliteCHEBI:15377 (ChEBI)
HSD17B4 dimerComplexREACT_17840 (Reactome)
HSD17B4ProteinP51659 (Uniprot-TrEMBL)
HSD3B7ProteinQ9H2F3 (Uniprot-TrEMBL)
NAD+MetaboliteCHEBI:15846 (ChEBI)
NADHMetaboliteCHEBI:16908 (ChEBI)
NADP+MetaboliteCHEBI:18009 (ChEBI)
NADPHMetaboliteCHEBI:16474 (ChEBI)
Na+MetaboliteCHEBI:29101 (ChEBI)
O2MetaboliteCHEBI:15379 (ChEBI)
PPiMetaboliteCHEBI:29888 (ChEBI)
PiMetaboliteCHEBI:18367 (ChEBI)
SCP2-1ProteinP22307-1 (Uniprot-TrEMBL)
SLC10A1ProteinQ14973 (Uniprot-TrEMBL)
SLC10A2ProteinQ12908 (Uniprot-TrEMBL)
SLC27A2ProteinO14975 (Uniprot-TrEMBL)
SLC27A5ProteinQ9Y2P5 (Uniprot-TrEMBL)
SLCO1A2ProteinP46721 (Uniprot-TrEMBL)
SLCO1B1ProteinQ9Y6L6 (Uniprot-TrEMBL)
SLCO1B3ProteinQ9NPD5 (Uniprot-TrEMBL)
THCA-CoAMetaboliteCHEBI:27505 (ChEBI)
THCAMetaboliteCHEBI:18402 (ChEBI)
TetraHCAMetaboliteCHEBI:63824 (ChEBI)
albumin bile salt and acid ComplexREACT_10829 (Reactome)
albumin bile salt complexComplexREACT_10145 (Reactome)
albumin cholate bile salt complexComplexREACT_10265 (Reactome)
bile salts and acids MetaboliteREACT_10444 (Reactome)
bile salts and acids complexed with FABP6ComplexREACT_10401 (Reactome)
bile salts and acids complexed with albuminComplexREACT_10309 (Reactome)
bile salts and acidsMetaboliteREACT_9533 (Reactome)
bile saltsMetaboliteREACT_10276 (Reactome)
bile saltsMetaboliteREACT_10834 (Reactome)
bile saltsMetaboliteREACT_11011 (Reactome)
chenodeoxycholate MetaboliteCHEBI:16755 (ChEBI)
chenodeoxycholate bile saltsMetaboliteREACT_10485 (Reactome)
chenodeoxycholoyl-CoAMetaboliteCHEBI:28701 (ChEBI)
cholate MetaboliteCHEBI:16359 (ChEBI)
cholate bile saltsMetaboliteREACT_10246 (Reactome)
cholate bile saltsMetaboliteREACT_10914 (Reactome)
cholate; chenodeoxycholateMetaboliteREACT_10869 (Reactome)
cholateMetaboliteCHEBI:16359 (ChEBI)
choloyl-CoA; chenodeoxycholoyl-CoAMetaboliteREACT_10852 (Reactome)
choloyl-CoAMetaboliteCHEBI:15519 (ChEBI)
glycine; taurineMetaboliteREACT_10219 (Reactome)
glycine; taurineMetaboliteREACT_10925 (Reactome)
glycochenodeoxycholate MetaboliteCHEBI:36274 (ChEBI)
glycocholate MetaboliteCHEBI:17687 (ChEBI)
propionyl CoAMetaboliteCHEBI:15539 (ChEBI)
taurochenodeoxycholate MetaboliteCHEBI:16525 (ChEBI)
taurochenodeoxycholate MetaboliteCHEBI:9407 (ChEBI)
taurocholate MetaboliteCHEBI:28865 (ChEBI)

Annotated Interactions

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SourceTargetTypeDatabase referenceComment
24-hydroxycholesterolArrowREACT_10129 (Reactome)
24-hydroxycholesterolREACT_10066 (Reactome)
25-hydroxycholesterolArrowREACT_10030 (Reactome)
25-hydroxycholesterolREACT_9950 (Reactome)
25ArrowREACT_10000 (Reactome)
25ArrowREACT_10003 (Reactome)
25ArrowREACT_10020 (Reactome)
25ArrowREACT_10049 (Reactome)
25ArrowREACT_10064 (Reactome)
25ArrowREACT_10106 (Reactome)
25ArrowREACT_10119 (Reactome)
25REACT_10003 (Reactome)
25REACT_10074 (Reactome)
25REACT_9952 (Reactome)
27-hydroxycholesterolArrowREACT_9953 (Reactome)
27HCHOLREACT_9994 (Reactome)
3,7,24THCA-CoAArrowREACT_10087 (Reactome)
3,7,24THCA-CoAArrowREACT_9970 (Reactome)
3,7,24THCAArrowREACT_9988 (Reactome)
3,7,24THCAREACT_10087 (Reactome)
3,7,24THCAREACT_9970 (Reactome)
3alpha,7alpha,12alpha,24ArrowREACT_10042 (Reactome)
3alpha,7alpha,12alpha,24REACT_10100 (Reactome)
3alpha,7alpha,12alpha-trihydroxy-5beta-cholest-24-one-CoAArrowREACT_10065 (Reactome)
3alpha,7alpha,12alpha-trihydroxy-5beta-cholest-24-one-CoAREACT_10113 (Reactome)
3alpha,7alpha,12alpha-trihydroxy-5beta-cholestan-27-alArrowREACT_10025 (Reactome)
3alpha,7alpha,12alpha-trihydroxy-5beta-cholestan-27-alREACT_10001 (Reactome)
3alpha,7alpha,24ArrowREACT_9976 (Reactome)
3alpha,7alpha,24REACT_9988 (Reactome)
3alpha,7alpha-dihydroxy-5beta-cholest-24-one-CoAArrowREACT_9967 (Reactome)
3alpha,7alpha-dihydroxy-5beta-cholest-24-one-CoAREACT_10103 (Reactome)
3alpha,7alpha-dihydroxy-5beta-cholestan-26-alArrowREACT_9960 (Reactome)
3alpha,7alpha-dihydroxy-5beta-cholestan-26-alREACT_10005 (Reactome)
4-cholesten-7alpha, 12alpha-diol-3-oneArrowREACT_10071 (Reactome)
4-cholesten-7alpha, 12alpha-diol-3-oneREACT_10048 (Reactome)
4-cholesten-7alpha,12alpha,24ArrowREACT_10016 (Reactome)
4-cholesten-7alpha,12alpha,24REACT_10131 (Reactome)
4-cholesten-7alpha,12alpha,27-triol-3-oneArrowREACT_10028 (Reactome)
4-cholesten-7alpha,12alpha,27-triol-3-oneREACT_10018 (Reactome)
4-cholesten-7alpha,24ArrowREACT_10090 (Reactome)
4-cholesten-7alpha,24REACT_10016 (Reactome)
4-cholesten-7alpha,24REACT_10086 (Reactome)
4-cholesten-7alpha,27-diol-3-oneArrowREACT_10115 (Reactome)
4-cholesten-7alpha,27-diol-3-oneREACT_10028 (Reactome)
4-cholesten-7alpha,27-diol-3-oneREACT_10102 (Reactome)
4-cholesten-7alpha-ol-3-oneArrowREACT_10067 (Reactome)
4-cholesten-7alpha-ol-3-oneREACT_10071 (Reactome)
4-cholesten-7alpha-ol-3-oneREACT_9972 (Reactome)
5beta-cholestan-3alpha, 7alpha, 12alpha-triolREACT_10009 (Reactome)
5beta-cholestan-3alpha, 7alpha-diolREACT_10075 (Reactome)
5beta-cholestan-3alpha,7alpha,12alpha,24ArrowREACT_10044 (Reactome)
5beta-cholestan-3alpha,7alpha,12alpha,24ArrowREACT_10104 (Reactome)
5beta-cholestan-3alpha,7alpha,12alpha,24REACT_10042 (Reactome)
5beta-cholestan-3alpha,7alpha,12alpha,24REACT_10104 (Reactome)
5beta-cholestan-3alpha,7alpha,12alpha,27-tetrolArrowREACT_10009 (Reactome)
5beta-cholestan-3alpha,7alpha,12alpha,27-tetrolArrowREACT_10038 (Reactome)
5beta-cholestan-3alpha,7alpha,12alpha,27-tetrolREACT_10025 (Reactome)
5beta-cholestan-3alpha,7alpha,12alpha-triolArrowREACT_10120 (Reactome)
5beta-cholestan-3alpha,7alpha,24ArrowREACT_10054 (Reactome)
5beta-cholestan-3alpha,7alpha,24ArrowREACT_10096 (Reactome)
5beta-cholestan-3alpha,7alpha,24REACT_10096 (Reactome)
5beta-cholestan-3alpha,7alpha,24REACT_9976 (Reactome)
5beta-cholestan-3alpha,7alpha,26-triolArrowREACT_10008 (Reactome)
5beta-cholestan-3alpha,7alpha,26-triolArrowREACT_10075 (Reactome)
5beta-cholestan-3alpha,7alpha,26-triolREACT_9960 (Reactome)
5beta-cholestan-3alpha,7alpha-diolArrowREACT_10068 (Reactome)
5beta-cholestan-7alpha,12alpha,24ArrowREACT_10131 (Reactome)
5beta-cholestan-7alpha,12alpha,24REACT_10044 (Reactome)
5beta-cholestan-7alpha,12alpha,27-triol-3-oneArrowREACT_10018 (Reactome)
5beta-cholestan-7alpha,12alpha,27-triol-3-oneREACT_10038 (Reactome)
5beta-cholestan-7alpha,24ArrowREACT_10086 (Reactome)
5beta-cholestan-7alpha,24REACT_10054 (Reactome)
5beta-cholestan-7alpha,27-diol-3-oneArrowREACT_10102 (Reactome)
5beta-cholestan-7alpha,27-diol-3-oneREACT_10008 (Reactome)
5beta-cholestan-7alpha-ol-3-oneArrowREACT_9972 (Reactome)
5beta-cholestan-7alpha-ol-3-oneREACT_10068 (Reactome)
5beta-cholesten-7alpha, 12alpha-diol-3-oneArrowREACT_10048 (Reactome)
5beta-cholesten-7alpha, 12alpha-diol-3-oneREACT_10120 (Reactome)
7 alpha-hydroxycholesterolArrowREACT_10052 (Reactome)
7 alpha-hydroxycholesterolREACT_10067 (Reactome)
ABCB11REACT_10101 (Reactome)
ABCC3REACT_10078 (Reactome)
ACOT8REACT_10061 (Reactome)
ACOX2 FADREACT_10003 (Reactome)
ACOX2 FADREACT_10074 (Reactome)
ADPArrowREACT_10078 (Reactome)
ADPArrowREACT_10101 (Reactome)
AKR1D1REACT_10018 (Reactome)
AKR1D1REACT_10048 (Reactome)
AKR1D1REACT_10086 (Reactome)
AKR1D1REACT_10102 (Reactome)
AKR1D1REACT_10131 (Reactome)
AKR1D1REACT_9972 (Reactome)
ALBArrowREACT_10072 (Reactome)
ALBArrowREACT_10091 (Reactome)
ALBArrowREACT_10124 (Reactome)
ALBArrowREACT_9958 (Reactome)
ALBREACT_10078 (Reactome)
AMACRREACT_10047 (Reactome)
AMACRREACT_10132 (Reactome)
AMACRREACT_9964 (Reactome)
AMACRREACT_9998 (Reactome)
AMPArrowREACT_10000 (Reactome)
AMPArrowREACT_10020 (Reactome)
AMPArrowREACT_10034 (Reactome)
AMPArrowREACT_10049 (Reactome)
AMPArrowREACT_10064 (Reactome)
AMPArrowREACT_10087 (Reactome)
AMPArrowREACT_10106 (Reactome)
AMPArrowREACT_10119 (Reactome)
AMPArrowREACT_9970 (Reactome)
ATPREACT_10000 (Reactome)
ATPREACT_10020 (Reactome)
ATPREACT_10034 (Reactome)
ATPREACT_10049 (Reactome)
ATPREACT_10064 (Reactome)
ATPREACT_10078 (Reactome)
ATPREACT_10087 (Reactome)
ATPREACT_10101 (Reactome)
ATPREACT_10106 (Reactome)
ATPREACT_10119 (Reactome)
ATPREACT_9970 (Reactome)
Aldo-keto reductase familyREACT_10008 (Reactome)
Aldo-keto reductase familyREACT_10038 (Reactome)
Aldo-keto reductase familyREACT_10044 (Reactome)
Aldo-keto reductase familyREACT_10054 (Reactome)
Aldo-keto reductase familyREACT_10068 (Reactome)
Aldo-keto reductase familyREACT_10120 (Reactome)
BAATREACT_9968 (Reactome)
BAATREACT_9974 (Reactome)
BAATREACT_9991 (Reactome)
CH25H REACT_10030 (Reactome)
CHOLREACT_10030 (Reactome)
CYP27A1REACT_10001 (Reactome)
CYP27A1REACT_10005 (Reactome)
CYP27A1REACT_10009 (Reactome)
CYP27A1REACT_10025 (Reactome)
CYP27A1REACT_10042 (Reactome)
CYP27A1REACT_10075 (Reactome)
CYP27A1REACT_10096 (Reactome)
CYP27A1REACT_10100 (Reactome)
CYP27A1REACT_10104 (Reactome)
CYP27A1REACT_9953 (Reactome)
CYP27A1REACT_9960 (Reactome)
CYP27A1REACT_9976 (Reactome)
CYP27A1REACT_9988 (Reactome)
CYP39A1REACT_10066 (Reactome)
CYP46A1REACT_10129 (Reactome)
Cholest-5-ene-3beta,7alpha,24ArrowREACT_10066 (Reactome)
Cholest-5-ene-3beta,7alpha,24REACT_10090 (Reactome)
Cholest-5-ene-3beta,7alpha,25-triolArrowREACT_9950 (Reactome)
Cholest-5-ene-3beta,7alpha,27-triolArrowREACT_9994 (Reactome)
Cholest-5-ene-3beta,7alpha,27-triolREACT_10115 (Reactome)
CholesterolREACT_10052 (Reactome)
CholesterolREACT_10129 (Reactome)
CholesterolREACT_9953 (Reactome)
CoA-SHArrowREACT_10061 (Reactome)
CoA-SHArrowREACT_9968 (Reactome)
CoA-SHArrowREACT_9974 (Reactome)
CoA-SHArrowREACT_9991 (Reactome)
CoA-SHREACT_10000 (Reactome)
CoA-SHREACT_10020 (Reactome)
CoA-SHREACT_10034 (Reactome)
CoA-SHREACT_10049 (Reactome)
CoA-SHREACT_10064 (Reactome)
CoA-SHREACT_10087 (Reactome)
CoA-SHREACT_10103 (Reactome)
CoA-SHREACT_10106 (Reactome)
CoA-SHREACT_10113 (Reactome)
CoA-SHREACT_10119 (Reactome)
CoA-SHREACT_9970 (Reactome)
Cytochrome P450 REACT_10016 (Reactome)
Cytochrome P450 REACT_10028 (Reactome)
Cytochrome P450 REACT_10052 (Reactome)
Cytochrome P450 REACT_10071 (Reactome)
Cytochrome P450 REACT_9950 (Reactome)
Cytochrome P450 REACT_9994 (Reactome)
DHCAArrowREACT_10005 (Reactome)
DHCAREACT_10064 (Reactome)
DHCAREACT_10119 (Reactome)
FABP6ArrowREACT_10078 (Reactome)
FABP6REACT_10050 (Reactome)
H+ArrowREACT_10025 (Reactome)
H+ArrowREACT_10065 (Reactome)
H+ArrowREACT_10067 (Reactome)
H+ArrowREACT_10090 (Reactome)
H+ArrowREACT_10115 (Reactome)
H+ArrowREACT_9960 (Reactome)
H+ArrowREACT_9967 (Reactome)
H+REACT_10001 (Reactome)
H+REACT_10005 (Reactome)
H+REACT_10008 (Reactome)
H+REACT_10009 (Reactome)
H+REACT_10016 (Reactome)
H+REACT_10018 (Reactome)
H+REACT_10028 (Reactome)
H+REACT_10030 (Reactome)
H+REACT_10038 (Reactome)
H+REACT_10042 (Reactome)
H+REACT_10044 (Reactome)
H+REACT_10048 (Reactome)
H+REACT_10052 (Reactome)
H+REACT_10054 (Reactome)
H+REACT_10066 (Reactome)
H+REACT_10068 (Reactome)
H+REACT_10071 (Reactome)
H+REACT_10075 (Reactome)
H+REACT_10086 (Reactome)
H+REACT_10096 (Reactome)
H+REACT_10100 (Reactome)
H+REACT_10102 (Reactome)
H+REACT_10104 (Reactome)
H+REACT_10120 (Reactome)
H+REACT_10129 (Reactome)
H+REACT_10131 (Reactome)
H+REACT_9950 (Reactome)
H+REACT_9953 (Reactome)
H+REACT_9972 (Reactome)
H+REACT_9976 (Reactome)
H+REACT_9988 (Reactome)
H+REACT_9994 (Reactome)
H2O2ArrowREACT_10003 (Reactome)
H2O2ArrowREACT_10074 (Reactome)
H2OArrowREACT_10000 (Reactome)
H2OArrowREACT_10001 (Reactome)
H2OArrowREACT_10005 (Reactome)
H2OArrowREACT_10009 (Reactome)
H2OArrowREACT_10016 (Reactome)
H2OArrowREACT_10028 (Reactome)
H2OArrowREACT_10030 (Reactome)
H2OArrowREACT_10034 (Reactome)
H2OArrowREACT_10042 (Reactome)
H2OArrowREACT_10052 (Reactome)
H2OArrowREACT_10066 (Reactome)
H2OArrowREACT_10071 (Reactome)
H2OArrowREACT_10075 (Reactome)
H2OArrowREACT_10087 (Reactome)
H2OArrowREACT_10096 (Reactome)
H2OArrowREACT_10100 (Reactome)
H2OArrowREACT_10104 (Reactome)
H2OArrowREACT_10106 (Reactome)
H2OArrowREACT_10129 (Reactome)
H2OArrowREACT_9950 (Reactome)
H2OArrowREACT_9953 (Reactome)
H2OArrowREACT_9970 (Reactome)
H2OArrowREACT_9976 (Reactome)
H2OArrowREACT_9988 (Reactome)
H2OArrowREACT_9994 (Reactome)
H2OREACT_10019 (Reactome)
H2OREACT_10061 (Reactome)
H2OREACT_10101 (Reactome)
H2OREACT_9952 (Reactome)
HSD17B4 dimerREACT_10019 (Reactome)
HSD17B4 dimerREACT_10065 (Reactome)
HSD17B4 dimerREACT_9952 (Reactome)
HSD17B4 dimerREACT_9967 (Reactome)
HSD3B7REACT_10067 (Reactome)
HSD3B7REACT_10090 (Reactome)
HSD3B7REACT_10115 (Reactome)
NAD+REACT_10065 (Reactome)
NAD+REACT_10067 (Reactome)
NAD+REACT_10090 (Reactome)
NAD+REACT_10115 (Reactome)
NAD+REACT_9967 (Reactome)
NADHArrowREACT_10065 (Reactome)
NADHArrowREACT_10067 (Reactome)
NADHArrowREACT_10090 (Reactome)
NADHArrowREACT_10115 (Reactome)
NADHArrowREACT_9967 (Reactome)
NADP+ArrowREACT_10001 (Reactome)
NADP+ArrowREACT_10005 (Reactome)
NADP+ArrowREACT_10008 (Reactome)
NADP+ArrowREACT_10009 (Reactome)
NADP+ArrowREACT_10016 (Reactome)
NADP+ArrowREACT_10018 (Reactome)
NADP+ArrowREACT_10028 (Reactome)
NADP+ArrowREACT_10030 (Reactome)
NADP+ArrowREACT_10038 (Reactome)
NADP+ArrowREACT_10042 (Reactome)
NADP+ArrowREACT_10044 (Reactome)
NADP+ArrowREACT_10048 (Reactome)
NADP+ArrowREACT_10052 (Reactome)
NADP+ArrowREACT_10054 (Reactome)
NADP+ArrowREACT_10066 (Reactome)
NADP+ArrowREACT_10068 (Reactome)
NADP+ArrowREACT_10071 (Reactome)
NADP+ArrowREACT_10075 (Reactome)
NADP+ArrowREACT_10086 (Reactome)
NADP+ArrowREACT_10096 (Reactome)
NADP+ArrowREACT_10100 (Reactome)
NADP+ArrowREACT_10102 (Reactome)
NADP+ArrowREACT_10104 (Reactome)
NADP+ArrowREACT_10120 (Reactome)
NADP+ArrowREACT_10129 (Reactome)
NADP+ArrowREACT_10131 (Reactome)
NADP+ArrowREACT_9950 (Reactome)
NADP+ArrowREACT_9953 (Reactome)
NADP+ArrowREACT_9972 (Reactome)
NADP+ArrowREACT_9976 (Reactome)
NADP+ArrowREACT_9988 (Reactome)
NADP+ArrowREACT_9994 (Reactome)
NADP+REACT_10025 (Reactome)
NADP+REACT_9960 (Reactome)
NADPHArrowREACT_10025 (Reactome)
NADPHArrowREACT_9960 (Reactome)
NADPHREACT_10001 (Reactome)
NADPHREACT_10005 (Reactome)
NADPHREACT_10008 (Reactome)
NADPHREACT_10009 (Reactome)
NADPHREACT_10016 (Reactome)
NADPHREACT_10018 (Reactome)
NADPHREACT_10028 (Reactome)
NADPHREACT_10030 (Reactome)
NADPHREACT_10038 (Reactome)
NADPHREACT_10042 (Reactome)
NADPHREACT_10044 (Reactome)
NADPHREACT_10048 (Reactome)
NADPHREACT_10052 (Reactome)
NADPHREACT_10054 (Reactome)
NADPHREACT_10066 (Reactome)
NADPHREACT_10068 (Reactome)
NADPHREACT_10071 (Reactome)
NADPHREACT_10075 (Reactome)
NADPHREACT_10086 (Reactome)
NADPHREACT_10096 (Reactome)
NADPHREACT_10100 (Reactome)
NADPHREACT_10102 (Reactome)
NADPHREACT_10104 (Reactome)
NADPHREACT_10120 (Reactome)
NADPHREACT_10129 (Reactome)
NADPHREACT_10131 (Reactome)
NADPHREACT_9950 (Reactome)
NADPHREACT_9953 (Reactome)
NADPHREACT_9972 (Reactome)
NADPHREACT_9976 (Reactome)
NADPHREACT_9988 (Reactome)
NADPHREACT_9994 (Reactome)
Na+ArrowREACT_10050 (Reactome)
Na+ArrowREACT_9958 (Reactome)
Na+REACT_10050 (Reactome)
Na+REACT_9958 (Reactome)
O2REACT_10001 (Reactome)
O2REACT_10003 (Reactome)
O2REACT_10005 (Reactome)
O2REACT_10009 (Reactome)
O2REACT_10016 (Reactome)
O2REACT_10028 (Reactome)
O2REACT_10030 (Reactome)
O2REACT_10042 (Reactome)
O2REACT_10052 (Reactome)
O2REACT_10066 (Reactome)
O2REACT_10071 (Reactome)
O2REACT_10074 (Reactome)
O2REACT_10075 (Reactome)
O2REACT_10096 (Reactome)
O2REACT_10100 (Reactome)
O2REACT_10104 (Reactome)
O2REACT_10129 (Reactome)
O2REACT_9950 (Reactome)
O2REACT_9953 (Reactome)
O2REACT_9976 (Reactome)
O2REACT_9988 (Reactome)
O2REACT_9994 (Reactome)
PPiArrowREACT_10000 (Reactome)
PPiArrowREACT_10020 (Reactome)
PPiArrowREACT_10034 (Reactome)
PPiArrowREACT_10049 (Reactome)
PPiArrowREACT_10064 (Reactome)
PPiArrowREACT_10087 (Reactome)
PPiArrowREACT_10106 (Reactome)
PPiArrowREACT_10119 (Reactome)
PPiArrowREACT_9970 (Reactome)
PiArrowREACT_10078 (Reactome)
PiArrowREACT_10101 (Reactome)
REACT_10000 (Reactome) TetraHCA (25(R) 3alpha,7alpha,12alpha,24(S)-tetrahydroxy-5beta-cholestanoate), coenzyme A, and ATP react to form the CoA conjugate of 25(R) TetraHCA, AMP, pyrophosphate and water. This cytosolic reaction is catalyzed by SLC27A5 (BACS). SLC27A2 (VLCS) also catalyzes this reaction; the relative contributions of the two enzymes to de novo bile acid synthesis in vivo are not certain (Mihalik et al. 2002).
REACT_10001 (Reactome) 3alpha,7alpha,12alpha-trihydroxy-5beta-cholestan-27-al, NADPH + H+, and O2 react to form 3alpha,7alpha,12alpha-trihydroxy-5beta-cholestanoate (THCA), NADP+, and H2O. This oxidation reaction occurs in the mitochondrial matrix, catalyzed by CYP27A1.
REACT_10003 (Reactome) 25(S) DHCA-CoA and O2 react to form 25(S) 3alpha,7alpha-dihydroxy-5beta-cholest-24-enoyl-CoA and H2O2. This dehydrogenation reaction occurs in the peroxisomal matrix. It is catalyzed by the FAD-containing peroxisomal enzyme branched chain acyl-CoA oxidase (ACOX2). The enzyme transfers electrons to molecular oxygen and hydrogen peroxide is produced as a byproduct.
REACT_10005 (Reactome) 3alpha, 7alpha-dihydroxy-5beta-cholestan-26-al, NADPH + H+, and O2 react to form 3alpha, 7alpha-dihydroxy-5beta-cholestanoate (DHCA), NADP+ and H2O. This oxidation reaction occurs in the mitochondrial matrix, catalyzed by CYP27A1.
REACT_10008 (Reactome) 5Beta-cholesten-7alpha,27-diol-3-one and NADPH + H+ form 5beta-cholestan-3alpha,7alpha,27-triol and NAPDP+. The reaction is catalyzed by 3alpha-hydroxysteroid dehydrogenase (AKR1C4), a cytosolic enzyme belonging to the aldo-keto reductase family (Dufort et al. 2001). Biochemical studies with rat proteins raise the possibility that other related enzymes may also carry out this reaction in vivo (Russell 2003).
REACT_10009 (Reactome) 5beta-cholestan-3alpha, 7alpha, 12alpha-triol, NADPH + H+, and O2 react to form 5beta-cholestan-3alpha, 7alpha, 12alpha, 27-tetrol + NADP+ + H2O. This reaction occurs in the mitochondrial matrix, catalyzed by CYP27A1.
REACT_10012 (Reactome) 25(R) DHCA-CoA is transported from the cytosol into the peroxisome. Indirect evidence suggests that a member of the ABC class of transporters mediates this reaction.
REACT_10015 (Reactome) 5beta-cholestan-3alpha,7alpha,12alpha,24(S)-tetrol is translocated from the cytosol to the mitochondrial matrix. The transporter that mediates its passage across the inner mitochondrial membrane is unknown: the StAR protein that performs this function for cholesterol at the start of steroid hormone biosynthesis is excluded as StAR is not expressed in liver. Other members of the START family of transporters are candidates, however (Russell 2003).
REACT_10016 (Reactome) 4-Cholesten-7alpha,24(S)-diol-3-one, NADPH + H+, and O2 form 4-cholesten-7-alpha,12-alpha,24(S)-triol-3-one + NADP+ + H2O. This reaction is catalyzed by sterol 12alpha hydroxylase (CYP8B1), an enzyme associated with the endoplasmic reticulum membrane. While the human gene has been cloned (Gafvels et al. 1999), its protein product has not been characterized, and the enzymatic properties of human CYP8B1 protein are inferred from those of its well-characterized rabbit homolog (Ishida et al. 1992).
REACT_10018 (Reactome) 4-Cholesten-7alpha,12alpha,27-triol-3-one and NADPH + H+ react to form 5beta-cholesten-7alpha,12alpha,27-triol-3-one + NADP+. This reaction is catalyzed by AKR1D1 (3-oxo-5-beta-steroid 4-dehydrogenase). AKR1D1 is localized to the cytosol, and in the course of the reaction its steroid substrate moves from the endoplasmic reticulum membrane to the cytosol. It is unclear whether this translocation results simply from its increased hydrophilicity or is mediated by the enzyme or another transport protein (Russell 2003).
REACT_10019 (Reactome) 3alpha,7alpha,12alpha-trihydroxy-5beta-cholest-24-enoyl-CoA (THCA-CoA) is hydrated to form (24R, 25R) 3alpha,7alpha,12alpha,24-tetrahydroxy-5beta-cholestanoyl-CoA. This reaction, catalyzed by the peroxisomal D-bifunctional enzyme (Huyghe et al. 2006), occurs in the peroxisomal matrix.
REACT_10020 (Reactome) THCA (25(R) 3alpha,7alpha,12alpha-trihydroxy-5beta-cholestanoate) , coenzyme A, and ATP react to form the CoA conjugate of 25(R) THCA, AMP, and pyrophosphate. This cytosolic reaction is catalyzed by SLC27A5 (BACS). SLC27A2 (VLCS) also catalyzes this reaction; the relative contributions of the two enzymes to de novo bile acid synthesis in vivo are not certain (Mihalik et al. 2002).
REACT_10025 (Reactome) 5beta-cholestan-3alpha, 7alpha, 12alpha, 27-tetrol and NADP+ react to form 3alpha, 7alpha, 12alpha-trihydroxy-5beta-cholestan-27-al and NADPH + H+. This oxidation reaction occurs in the mitochondrial matrix, catalyzed by CYP27A1.
REACT_10028 (Reactome) 4-Cholesten-7alpha,27-diol-3-one, NADPH + H+, and O2 form 4-Cholesten-7alpha,12alpha,27-triol-3-one + NADP+ + H2O. This reaction is catalyzed by sterol 12alpha hydroxylase (CYP8B1), an enzyme associated with the endoplasmic reticulum membrane. While the human gene has been cloned (Gafvels et al. 1999), its protein product has not been characterized, and the enzymatic properties of human CYP8B1 protein are inferred from those of its well-characterized rabbit homolog (Ishida et al. 1992).
REACT_10030 (Reactome) The microsomal enzyme cholesterol 25-hydroxylase is a member of a lipid metabolizing enzyme family that utilizes oxygen and diiron-oxygen cofactor to hydroxylate, desaturate, epoxidate and acetylate substrates.
REACT_10034 (Reactome) Cholate or chenodeoxycholate, coenzyme A, and ATP react to form their CoA conjugates, AMP, pyrophosphate and water. This reaction is catalyzed by SLC27A5 (BACS) associated with the endoplasmic reticulum membrane, but the substrates and products are cytosolic (Mihalik et al. 2002).
REACT_10038 (Reactome) 5Beta-cholesten-7alpha,12a,27-triol-3-one and NADPH + H+ form 5beta-cholestan-3alpha,7alpha,12a,27-tetrol and NAPDP+. The reaction is catalyzed by 3alpha-hydroxysteroid dehydrogenase (AKR1C4), a cytosolic enzyme belonging to the aldo-keto reductase family (Dufort et al. 2001). Biochemical studies with rat proteins raise the possibility that other related enzymes may also carry out this reaction in vivo (Russell 2003).
REACT_10042 (Reactome) 5beta-cholestan-3alpha,7alpha,12alpha,24(S),27-pentol, NADPH + H+, and O2 react to form 3alpha,7alpha,12alpha,24(S)-tetrahydroxy-5beta-cholestan-27-al, NADP+, and H2O. This oxidation reaction occurs in the mitochondrial matrix, catalyzed by CYP27A1.
REACT_10044 (Reactome) 5Beta-cholesten-7alpha,12alpha,24(S)-triol-3-one and NADPH + H+ form 5beta-cholestan-3alpha,7alpha,12alpha,24(S)-tetrol and NAPDP+. The reaction is catalyzed by 3alpha-hydroxysteroid dehydrogenase (AKR1C4), a cytosolic enzyme belonging to the aldo-keto reductase family (Dufort et al. 2001). Biochemical studies with rat proteins raise the possibility that other related enzymes may also carry out this reaction in vivo (Russell 2003).
REACT_10047 (Reactome) The isomerization of 25(R) DHCA-CoA to 25(S) DHCA-CoA, catalyzed by 2-methylacyl-CoA racemase, occurs in the peroxisomal matrix.
REACT_10048 (Reactome) 4-Cholesten-7alpha, 12alpha-diol-3-one and NADPH + H+ react to form 5beta-cholesten-7alpha,12alpha-diol-3-one + NADP+. This reaction is catalyzed by AKR1D1 (3-oxo-5-beta-steroid 4-dehydrogenase). AKR1D1 is localized to the cytosol, and in the course of the reaction its steroid substrate moves from the endoplasmic reticulum membrane to the cytosol. It is unclear whether this translocation results simply from its increased hydrophilicity or is mediated by the enzyme or another transport protein (Russell 2003).
REACT_10049 (Reactome) THCA (25(R) 3alpha,7alpha,12alpha-trihydroxy-5beta-cholestanoate), coenzyme A, and ATP react to form the CoA conjugate of 25(R) THCA, AMP, and pyrophosphate. This cytosolic reaction is catalyzed by SLC27A2 (VLCS). SLC27A5 (BACS) also catalyzes this reaction; the relative contributions of the two enzymes to de novo bile acid synthesis in vivo are not certain (Mihalik et al. 2002).
REACT_10050 (Reactome) A molecule of extracellular bile salt or bile acid (cholate, chenodeoxycholate, or their glycine or taurine conjugates) and a sodium ion are transported into the cytosol, mediated by ASBT (apical sodium-dependent bile acid transporter; SLC10A2) in the plasma membrane. Within the cytosol, bile salts and acids are bound to a carrier protein, FABP6 (I-BABP) (Fujita et al. 1995). Studies in a rabbit model system suggest that translocation and FABP6 binding occur as a single concerted event (Kramer et al. 1995). In the body, ASBT is expressed on the apical surfaces of enterocytes, and this reaction is the first step in the process by which bile salts and acids are reaborbed from the intestinal lumen and returned to the liver (Kullak-Ublick et al. 2004; Trauner and Boyer 2002).
REACT_10052 (Reactome) Cholesterol, NADPH + H+, and O2 form 7alpha-cholesterol (5-cholesten-3beta, 7alpha-diol), NADP+,and H2O, in a reaction catalyzed by CYP7A1 (cholesterol 7alpha-hydroylase) in the endoplasmic reticulum membrane. In the body, this enzyme is expressed only in liver, and its expression is tightly regulated at the level of transcription to determine the overall rate of bile acid and bile salt production (Russell 2003; Pullinger et al. 2002).
REACT_10054 (Reactome) 5Beta-cholesten-7alpha,24(S)-diol-3-one and NADPH + H+ form 5beta-cholestan-3alpha,7alpha,24(S)-triol and NAPDP+. The reaction is catalyzed by 3alpha-hydroxysteroid dehydrogenase (AKR1C4), a cytosolic enzyme belonging to the aldo-keto reductase family (Dufort et al. 2001). Biochemical studies with rat proteins raise the possibility that other related enzymes may also carry out this reaction in vivo (Russell 2003).
REACT_10057 (Reactome) 27-hydroxycholesterol is transported from the extracellular space to the endoplasmic reticulum. In humans, this event is the means by which the molecule, generated from cholesterol in the brain, is taken up by liver cells for conversion to bile acids and bile salts (Babiker et al. 1999; Bjorkhem et al. 1994). While transport proteins are likely to play a role in this process, these proteins have not been identified.
REACT_10061 (Reactome) Choloyl-CoA and water react to form cholate and CoASH. This reaction, catalyzed by acyl-coenzyme A thioesterase 8, occurs in the peroxisomal matrix (Jones et al. 1999; Hunt et al. 2002). While bile salts are the major product of the de novo biosynthetic pathway in the normal human liver, bile acids are major feedback regulators of this pathway and this hydrolysis reaction is thought to play a role in generating them (Russell 2003).
REACT_10064 (Reactome) DHCA (25(R) 3alpha,7alpha-dihydroxy-5beta-cholestanoate) , coenzyme A, and ATP react to form the CoA conjugate of 25(R) DHCA, AMP, and pyrophosphate. This cytosolic reaction is catalyzed by SLC27A5 (BACS). SLC27A2 (VLCS) also catalyzes this reaction; the relative contributions of the two enzymes to de novo bile acid synthesis in vivo are not certain (Mihalik et al. 2002).
REACT_10065 (Reactome)
REACT_10066 (Reactome) 24-hydroxycholesterol, NADPH + H+, and O2 react to form cholest-5-ene-3beta,7alpha,24-triol, NADP+, and H2O. This hydroxylation reaction is catalyzed by CYP39A1 in the membrane of the endoplasmic reticulum. In the body, expression of CYP39A1 is restricted to the liver.
REACT_10067 (Reactome) 7alpha-hydroxycholesterol and NAD+ react to form 4-cholesten-7alpha-ol-3-one, NADH, and H+, in a reaction catalyzed by HSD3B7 (3 beta-hydroxysteroid dehydrogenase type 7) in the endoplasmic reticulum membrane. Its function in vivo has been confirmed in studies of patients with defects in bile acid synthesis (Schwarz et al. 2000).
REACT_10068 (Reactome) 5Beta-cholesten-7alpha-ol-3-one and NADPH + H+ form 5beta-cholestan-3alpha,7alpha-diol and NAPDP+. The reaction is catalyzed by 3alpha-hydroxysteroid dehydrogenase (AKR1C4), a cytosolic enzyme belonging to the aldo-keto reductase family (Dufort et al. 2001). Biochemical studies with rat proteins raise the possibility that other related enzymes may also carry out this reaction in vivo (Russell 2003).
REACT_10071 (Reactome) 7-Alpha-hydroxycholest-4-en-3-one, NADPH + H+, and O2 form 7-alpha,12-alpha-dihydroxycholest-4-en-3-one + NADP+ + H2O. This reaction is catalyzed by sterol 12alpha hydroxylase (CYP8B1), an enzyme associated with the endoplasmic reticulum membrane. While the human gene has been cloned (Gafvels et al. 1999), its protein product has not been characterized, and the enzymatic properties of human CYP8B1 protein are inferred from those of its well-characterized rabbit homolog (Ishida et al. 1992).
REACT_10072 (Reactome) A molecule of extracellular bile salt (glyco- or taurocholate or taurochenodeoxycholate) or bile acid (cholate or chenodeoxycholate) is transported into the cytosol, mediated by OATP-A (SLCO1A2) in the plasma membrane. Bile salts and acids exist in the blood as complexes with serum albumin, and their uptake by OATP-A must involve disruption of this complex, but the molecular mechanism coupling release of a bile salt or acid from albumin to its uptake by OATP-A is unknown. In the body, OATP-A is expressed only at low levels on the basolateral surfaces of hepatocytes and may play only a minor role in the uptake of bile salts and acids by the liver (Kullak-Ublick et al. 2004; Trauner and Boyer 2002).
REACT_10073 (Reactome) TetraHCA (3alpha,7alpha,12alpha,24(S)-tetrahydroxy-5beta-cholestanoate) is translocated from the mitochondrial matrix to the cytosol. The transporter that mediates its passage across the inner mitochondrial membrane has not been identified (Russell 2003). VLCS (SLC27A2), one of the enzymes that catalyzes CoA conjugation of TetraHCA, may also be present in peroxisomes, and Mihalik et al. (2002) have hypothesized that TetraHCA could be translocated unchanged from the mitochondrial matrix to the peroxisomal matrix and undergo conjugation there.
REACT_10074 (Reactome) 25(S) THCA-CoA and O2 react to form 3alpha,7alpha,12alpha-trihydroxy-5beta-cholest-24-enoyl-CoA (THCA-CoA) and H2O2. This dehydrogenation reaction occurs in the peroxisomal matrix. It is catalyzed by the FAD-containing peroxisomal enzyme branched chain acyl-CoA oxidase (ACOX2). The enzyme transfers electrons to molecular oxygen and hydrogen peroxide is produced as a byproduct.
REACT_10075 (Reactome) 5beta-cholestan-3alpha, 7alpha-diol, NADPH + H+, and O2 react to form 5beta-cholestan-3alpha, 7alpha, 26-triol + NADP+ + H2O. This reaction occurs in the mitochondrial matrix, catalyzed by CYP27A1.
REACT_10078 (Reactome) A molecule of glycocholate, taurocholate, or taurochenodeoxycholate is transported from the cytosol to the extracellular space, coupled to the hydrolysis of a molecule of cytosolic ATP to ADP and orthophosphate. This reaction is mediated by ABCB3 (MRP3). In the body, this reaction mediates the release of bile salts from enterocytes into the blood (Kullak-Ublick et al. 2004; Trauner and Boyer 2002). In the cytosol, bile salts and acids are bound to a carrier protein, FABP6 (I-BABP) (Fujita et al. 1995), and in the blood these molecules are complexed with albumin. The mechanisms by which transport across the plasma membrane is coupled to release FABP6 and binding to albumin are unknown, so the entire process is annotated as a single concerted event.
REACT_10080 (Reactome) 5beta-cholestan-3alpha,7alpha,27-triol is transported from the cytosol to the mitochondrial matrix. The transporter that mediates its passage across the inner mitochondrial membrane is unknown: the StAR protein that performs this function for cholesterol at the start of steroid hormone biosynthesis is excluded as StAR is not expressed in liver. Other members of the START family of transporters are candidates, however (Russell 2003).
REACT_10085 (Reactome) 5beta-cholestan-3alpha, 7alpha-diol is transported from the cytosol to the mitochondrial matrix. The transporter that mediates its passage across the inner mitochondrial membrane is unknown: the StAR protein that performs this function for cholesterol at the start of steroid hormone biosynthesis is excluded as StAR is not expressed in liver. Other members of the START family of transporters are candidates, however (Russell 2003).
REACT_10086 (Reactome) 4-Cholesten-7alpha,24(S)-diol-3-one, NADPH, and H+ react to form 5beta-cholestan-7alpha,24(S)-diol-3-one and NADP+. This reaction is catalyzed by AKR1D1 (3-oxo-5-beta-steroid 4-dehydrogenase). AKR1D1 is localized to the cytosol, and in the course of the reaction its steroid substrate moves from the endoplasmic reticulum membrane to the cytosol. It is unclear whether this translocation results simply from its increased hydrophilicity or is mediated by the enzyme or another transport protein (Russell 2003).
REACT_10087 (Reactome) 3,7,24THCA (25(R) 3alpha,7alpha,24(S)-trihydroxy-5beta-cholestanoate), coenzyme A, and ATP react to form the CoA conjugate of 3,7,24THCA, AMP, pyrophosphate and water. This cytosolic reaction is catalyzed by SLC27A5 (BACS). SLC27A2 (VLCS) also catalyzes this reaction; the relative contributions of the two enzymes to de novo bile acid synthesis in vivo are not certain (Mihalik et al. 2002).
REACT_10089 (Reactome) THCA (3alpha,7alpha,12alpha-trihydroxy-5beta-cholestanoate) is translocated from the mitochondrial matrix to the cytosol. The transporter that mediates its passage across the inner mitochondrial membrane has not been identified. In particular, despite the structural and functional similarities between THCA and long chain fatty acids, searches for carnitine - THCA have failed (Russell 2003). VLCS (SLC27A2), one of the enzymes that catalyzes CoA conjugation of THCA may also be present in peroxisomes, and Mihalik et al. (2002) have hypothesized that THCA could be translocated unchanged from the mitochondrial matrix to the peroxisomal matrix and undergo conjugation there.
REACT_10090 (Reactome) Cholest-5-ene-3beta,7alpha,24(S)-triol and NAD+ react to form 4-cholesten-7alpha,24(S)-diol-3-one and NADH + H+, catalyzed by HSD3B7 (3 beta-hydroxysteroid dehydrogenase type 7) in the endoplasmic reticulum membrane. Its function in vivo has been confirmed in studies of patients with defects in bile acid synthesis (Schwarz et al. 2000).
REACT_10091 (Reactome) A molecule of extracellular glycocholate or taurocholate is transported into the cytosol, mediated by OATP-C (SLCO1B1) in the plasma membrane. Glyco- and taurocholate exist in the blood as complexes with serum albumin, and its uptake by OATP-C must involve disruption of this complex, but the molecular mechanism coupling disruption and uptake is unknown. In the body, OATP-C is expressed on the basolateral surfaces of hepatocytes and may play a role in the uptake of glyco- and taurocholate by the liver under physiological conditions (Kullak-Ublick et al. 2004; Trauner and Boyer 2002).
REACT_10096 (Reactome) 5beta-cholestan-3alpha,7alpha,24(S)-triol, NADPH + H+, and O2 react to form 5beta-cholestan-3alpha,7alpha,24(S),27-tetrol, NADP+, and H2O. This reaction occurs in the mitochondrial matrix, catalyzed by CYP27A1.
REACT_10097 (Reactome) 5beta-cholestan-3alpha,7alpha,12alpha-triol is translocated from the cytosol to the mitochondrial matrix. The transporter that mediates its passage across the inner mitochondrial membrane is unknown: the StAR protein that performs this function for cholesterol at the start of steroid hormone biosynthesis is excluded as StAR is not expressed in liver. Other members of the START family of transporters are candidates, however (Russell 2003).
REACT_10099 (Reactome) 24-hydroxycholesterol is transported from the endoplasmic reticulum to the extracellular space. In humans, this event is the major source of 24-hydroxycholesterol in the blood and is the means by which the molecule, generated from cholesterol in the brain, is transported to the liver for conversion to bile acids and bile salts. While transport proteins are likely to play a role in this process, and the 24-hydroxycholesterol is likely to occur as part of a lipoprotein complex in the blood, the relevant proteins have not been identified (Lutjohann et al. 1996; Bjorkhem et al. 1998).
REACT_10100 (Reactome) 3alpha,7alpha,12alpha,24(S)-tetrahydroxy-5beta-cholestan-27-al, NADPH + H+, and O2 react to form 3alpha,7alpha,12alpha,24(S)-tetrahydroxy-5beta-cholestanoate (TetraHCA), NADP+, and H2O. This oxidation reaction occurs in the mitochondrial matrix, catalyzed by CYP27A1.
REACT_10101 (Reactome) A molecule of glycocholate, glycochenodeoxycholate, taurocholate, or taurochenodeoxycholate is transported from the cytosol to the extracellular space, coupled to the hydrolysis of a molecule of cytosolic ATP to ADP and orthophosphate. This reaction is mediated by ABCB11 (bile salt export pump). In the body, this reaction mediates the release of bile salts from the liver cells into the bile (Kullak-Ublick et al. 2004); the role of ABCB11 in the reaction is confirmed by the observed failure of bile salt export in patients in whom the transporter is defective (Noe et al. 2005).
REACT_10102 (Reactome) 4-Cholesten-7alpha,27-diol-3-one, NADPH, and H+ react to form 5beta-cholestan-7alpha,27-diol-3-one and NADP+. This reaction is catalyzed by AKR1D1 (3-oxo-5-beta-steroid 4-dehydrogenase). AKR1D1 is localized to the cytosol, and in the course of the reaction its steroid substrate moves from the endoplasmic reticulum membrane to the cytosol. It is unclear whether this translocation results simply from its increased hydrophilicity or is mediated by the enzyme or another transport protein (Russell 2003).
REACT_10103 (Reactome) 3alpha,7alpha-dihydroxy-5beta-cholan-24-one-CoA and CoASH react to form chenodeoxycholoyl-CoA (3alpha,7alpha-dihydroxy-5beta-cholan-24-one-CoA) and propionyl CoA. This reaction, in the peroxisomal matrix, is catalyzed by peroxisomal thiolase 2 (sterol carrier protein 2).
REACT_10104 (Reactome) 5beta-cholestan-3alpha,7alpha,12alpha,24(S)-tetrol, NADPH + H+, and O2 react to form 5beta-cholestan-3alpha,7alpha,12alpha,24(S),27-pentol, NADP+, and H2O. This reaction occurs in the mitochondrial matrix, catalyzed by CYP27A1.
REACT_10105 (Reactome) 25(R) THCA-CoA is transported from the cytosol into the peroxisome. Indirect evidence suggests that a member of the ABC class of transporters mediates this reaction.
REACT_10106 (Reactome) TetraHCA (25(R) 3alpha,7alpha,12alpha,24(S)-tetrahydroxy-5beta-cholestanoate), coenzyme A, and ATP react to form the CoA conjugate of 25(R) TetraHCA, AMP, pyrophosphate and water. This cytosolic reaction is catalyzed by SLC27A2 (VLCS). SLC27A5 (BACS) also catalyzes this reaction; the relative contributions of the two enzymes to de novo bile acid synthesis in vivo are not certain (Mihalik et al. 2002).
REACT_10107 (Reactome) DHCA (3alpha, 7alpha-dihydroxy-5beta-cholestanoate) is translocated from the mitochondrial matrix to the cytosol. The transporter that mediates its passage across the inner mitochondrial membrane has not been identified. In particular, despite the structural and functional similarities between DHCA and long chain fatty acids, searches for carnitine - DHCA have failed (Russell 2003). VLCS (SLC27A2), one of the enzymes that catalyzes CoA conjugation of DHCA, may also be present in peroxisomes, and Mihalik et al. (2002) have hypothesized that DHCA could be translocated unchanged from the mitochondrial matrix to the peroxisomal matrix and undergo conjugation there.
REACT_10112 (Reactome) 5beta-cholestan-3alpha,7alpha,24(S)-triol is transported from the cytosol to the mitochondrial matrix. The transporter that mediates its passage across the inner mitochondrial membrane is unknown: the StAR protein that performs this function for cholesterol at the start of steroid hormone biosynthesis is excluded as StAR is not expressed in liver. Other members of the START family of transporters are candidates, however (Russell 2003).
REACT_10113 (Reactome) 3alpha,7alpha,12alpha-trihydroxy-5beta-cholan-24-one-CoA and CoASH react to form choloyl-CoA (3alpha,7alpha,12alpha-trihydroxy-5beta-cholan-24-one-CoA) and propionyl CoA. This reaction, in the peroxisomal matrix, is catalyzed by peroxisomal thiolase 2 (sterol carrier protein 2).
REACT_10115 (Reactome) Cholest-5-ene-3beta,7alpha,27-triol and NAD+ react to form 4-cholesten-7alpha,27-diol-3-one and NADH + H+, in a reaction catalyzed by HSD3B7 (3 beta-hydroxysteroid dehydrogenase type 7) in the endoplasmic reticulum membrane. Its function in vivo has been confirmed in studies of patients with defects in bile acid synthesis (Schwarz et al. 2000).
REACT_10118 (Reactome) 3,7,24THCA-CoA is transported from the cytosol into the peroxisome.
REACT_10119 (Reactome) DHCA (25(R) 3alpha,7alpha-dihydroxy-5beta-cholestanoate), coenzyme A, and ATP react to form the CoA conjugate of 25(R) DHCA, AMP, and pyrophosphate. This cytosolic reaction is catalyzed by SLC27A2 (VLCS). SLC27A5 (BACS) also catalyzes this reaction; the relative contributions of the two enzymes to de novo bile acid synthesis in vivo are not certain (Mihalik et al. 2002).
REACT_10120 (Reactome) 5Beta-cholesten-7alpha,12alpha-diol-3-one and NADPH + H+ form 5beta-cholestan-3alpha,7alpha,12alpha-triol and NAPDP+. The reaction is catalyzed by 3alpha-hydroxysteroid dehydrogenase (AKR1C4), a cytosolic enzyme belonging to the aldo-keto reductase family (Dufort et al. 2001). Biochemical studies with rat proteins raise the possibility that other related enzymes may also carry out this reaction in vivo (Russell 2003).
REACT_10121 (Reactome) 25(R) TetraHCA-CoA is transported from the cytosol into the peroxisome.
REACT_10124 (Reactome) A molecule of extracellular glycocholate or taurocholate is transported into the cytosol, mediated by OATP-8 (SLCO1B3) in the plasma membrane. Glycocholate and taurocholate exist in the blood as complexes with serum albumin, and their uptake by OATP-8 must involve disruption of these complexes, but the molecular mechanism coupling disruption and uptake is unknown. In the body, OATP-8 is expressed on the basolateral surfaces of hepatocytes and may play a role in the uptake of glycocholate and taurocholate by the liver under physiological conditions (Kullak-Ublick et al. 2004; Trauner and Boyer 2002).
REACT_10129 (Reactome) Cholesterol, NADPH + H+, and O2 react to form 24-hydroxycholesterol , NADP+, and H2O. This reaction is catalyzed by CYP46A1 in the endoplasmic reticulum membrane. In the body, this enzyme is expressed predominantly in the brain and is thought to play a major role in cholesterol turnover there (Javitt 2002).
REACT_10130 (Reactome) Cholate is translocated from the peroxisomal matrix to the cytosol. The transporter that mediates this event is unknown.
REACT_10131 (Reactome) 4-cholesten-7alpha,12alpha,24(S)-triol-3-one and NADPH + H+ react to form 5beta-cholesten-7alpha,12alpha,24(S)-triol-3-one and NADP+. This reaction is catalyzed by AKR1D1 (3-oxo-5-beta-steroid 4-dehydrogenase). AKR1D1 is localized to the cytosol, and in the course of the reaction its steroid substrate moves from the endoplasmic reticulum membrane to the cytosol. It is unclear whether this translocation results simply from its increased hydrophilicity or is mediated by the enzyme or another transport protein (Russell 2003).
REACT_10132 (Reactome) The isomerization of 25(R) TetraHCA-CoA to (24R, 25R) 3alpha,7alpha,12alpha,24-tetrahydroxy-5beta-cholestanoyl-CoA, catalyzed by 2-methylacyl-CoA racemase, occurs in the peroxisomal matrix.
REACT_9950 (Reactome) 25-hydroxycholesterol is 7alpha-hydroxylated to cholest-5-ene-3beta,7alpha,25-triol by CYP7B1.
REACT_9952 (Reactome) 25(S) 3alpha,7alpha-dihydroxy-5beta-cholest-24-enoyl-CoA is hydrated to form (24R, 25R) 3alpha,7alpha,24-trihydroxy-5beta-cholestanoyl-CoA. This reaction, catalyzed by the peroxisomal D-bifunctional enzyme (Huyghe et al. 2006), occurs in the peroxisomal matrix.
REACT_9953 (Reactome) Cholesterol, NADPH + H+, and O2 react to form 27-hydroxycholesterol, H2O, and NADP+, in the mitochondrial matrix, catalyzed by CYP27A1. 27-Hydroxycholesterol is the most abundant oxysterol in the plasma in humans; its formation is thought to play a central role in the mobilization of cholesterol from non-hepatic tissues.
REACT_9958 (Reactome) A molecule of extracellular bile salt (glyco- or taurocholate, or glyco- or taurochenodeoxycholate) and two sodium ions are transported into the cytosol, mediated by NTCP (Na+ / taurocholate cotransporter) in the plasma membrane. Bile salts exist in the blood as complexes with serum albumin, and their uptake by NTCP must involve disruption of this complex, but the molecular mechanism of the coupling of the release of a bile salt from albumin to its uptake by NTCP is unknown. In the body, NTCP is expressed on the basolateral surfaces of hepatocytes, and this reaction is the major route by which bile salts reaborbed from the intestinal lumen into the portal circulation are recovered by the liver (Kullak-Ublick et al. 2004; Trauner and Boyer 2002).
REACT_9960 (Reactome) 5beta-cholestan-3alpha, 7alpha, 26-triol and NADP+ react to form 3alpha, 7alpha-dihydroxy-5beta-cholestan-26-al and NADPH + H+. This oxidation reaction occurs in the mitochondrial matrix, catalyzed by CYP27A1.
REACT_9964 (Reactome) The isomerization of 3,7,24THCA-CoA to (24R, 25R) 3alpha,7alpha,24-trihydroxy-5beta-cholestanoyl-CoA, catalyzed by 2-methylacyl-CoA racemase, occurs in the peroxisomal matrix.
REACT_9965 (Reactome) The bile salts glycocholate, glycochenodeoxycholate, taurocholate, and taurochenodeoxycholate are translocated from the peroxisomal matrix to the cytosol. The transporter that mediates this process is unknown (Russell 2003).
REACT_9967 (Reactome)
REACT_9968 (Reactome) Chenodeoxycholoyl CoA reacts with glycine or taurine to form glycochenodeoxycholate or taurochenodeoxycholate, releasing CoASH. This reaction, which completes the de novo synthesis of bile salts from cholesterol in vivo, is catalyzed by BAAT (Bile acid CoA:amino acid N-acyltransferase - Falany et al. 1994) and occurs in the peroxisomal matrix (Solaas et al. 2000; Mihalik et al. 2002). In vivo, the relative amounts of glycochenodeoxycholate and taurochenodeoxycholate synthesized appear to be determined solely by the intracellular abundances of glycine and taurine (Russell 2003).
REACT_9970 (Reactome) 3,7,24THCA (25(R) 3alpha,7alpha,24(S)-trihydroxy-5beta-cholestanoate), coenzyme A, and ATP react to form the CoA conjugate of 3,7,24THCA, AMP, pyrophosphate and water. This cytosolic reaction is catalyzed by SLC27A2 (VLCS). SLC27A5 (BACS) also catalyzes this reaction; the relative contributions of the two enzymes to de novo bile acid synthesis in vivo are not certain (Mihalik et al. 2002).
REACT_9972 (Reactome) 4-Cholesten-7alpha-ol-3-one, NADPH, and H+ react to form 5beta-cholestan-7alpha-ol-3-one and NADP+. This reaction is catalyzed by AKR1D1 (3-oxo-5-beta-steroid 4-dehydrogenase). AKR1D1 is localized to the cytosol, and in the course of the reaction its steroid substrate moves from the endoplasmic reticulum membrane to the cytosol. It is unclear whether this translocation results simply from its increased hydrophilicity or is mediated by the enzyme or another transport protein (Russell 2003).
REACT_9974 (Reactome) Choloyl CoA reacts with glycine or taurine to form glycocholate or taurocholate, releasing CoASH. This reaction, which completes the de novo synthesis of bile salts from cholesterol in vivo, is catalyzed by BAAT (Bile acid CoA:amino acid N-acyltransferase - Falany et al. 1994) and occurs in the peroxisomal matrix (Solaas et al. 2000; Mihalik et al. 2002). In vivo, the relative amounts of glycocholate and taurocholate synthesized appear to be determined solely by the intracellular abundances of glycine and taurine (Russell 2003).
REACT_9976 (Reactome) 5beta-cholestan-3alpha,7alpha,24(S),27-tetrol, NADPH + H+, and O2 react to form 3alpha,7alpha,24(S)-trihydroxy-5beta-cholestan-27-al, NADP+, and H2O. This oxidation reaction occurs in the mitochondrial matrix, catalyzed by CYP27A1.
REACT_9979 (Reactome) 24-hydroxycholesterol is transported from the extracellular space to the endoplasmic reticulum. In humans, this event is the means by which the molecule, generated from cholesterol in the brain, is taken up by liver cells for conversion to bile acids and bile salts. While transport proteins are likely to play a role in this process, these proteins have not been identified (Lutjohann et al. 1996; Bjorkhem et al. 1998).
REACT_9984 (Reactome) 27-hydroxycholesterol is transported from the mitochondrial matrix to the extracellular space. In humans, this event is the major source of 27-hydroxycholesterol in the blood and is the means by which the molecule, generated from cholesterol in a variety of cell types, notably macrophages, is transported to the liver for conversion to bile acids and bile salts (Babiker et al. 1999; Bjorkhem et al. 1994). While transport proteins are likely to play a role in this process, the relevant proteins have not been identified.
REACT_9985 (Reactome) 5beta-cholestan-3alpha,7alpha,12alpha,27-tetrol is translocated from the cytosol to the mitochondrial matrix. The transporter that mediates its passage across the inner mitochondrial membrane is unknown: the StAR protein that performs this function for cholesterol at the start of steroid hormone biosynthesis is excluded as StAR is not expressed in liver. Other members of the START family of transporters are candidates, however (Russell 2003).
REACT_9988 (Reactome) 3alpha,7alpha,24(S)-trihydroxy-5beta-cholestan-27-al, NADPH + H+, and O2 react to form 3alpha,7alpha,24(S)-trihydroxy-5beta-cholestanoate (3,7,24THCA), NADP+, and H2O. This oxidation reaction occurs in the mitochondrial matrix, catalyzed by CYP27A1.
REACT_9991 (Reactome) Cytosolic bile acid-CoA conjugates (choloyl-CoA; chenodeoxycholoyl-CoA) react with the amino acids glycine and taurine, generating the corresponding bile salts and coenzyme A, catalyzed by BAAT (bile acid-CoA:amino acid N-acetyltransferase). In the body, this reaction occurs in hepatocytes and is the means by which bile acids recovered from the intestine are converted to bile salts before being released again into the bile (Kullak-Ublick et al. 2004; Trauner and Boyer 2002).
REACT_9992 (Reactome) 3,7,24THCA (3alpha,7alpha,24(S)-trihydroxy-5beta-cholestanoate) is translocated from the mitochondrial matrix to the cytosol. The transporter that mediates its passage across the inner mitochondrial membrane has not been identified (Russell 2003). VLCS (SLC27A2), one of the enzymes that catalyzes CoA conjugation of 3,7,24THCA, may also be present in peroxisomes, and Mihalik et al. (2002) have hypothesized that 3,7,24THCA could be translocated unchanged from the mitochondrial matrix to the peroxisomal matrix and undergo conjugation there.
REACT_9994 (Reactome) 27-hydroxycholesterol, NADPH + H+, and O2 react to form cholest-5-ene-3beta,7alpha,27-triol, H2O, and NADP+. This reaction is catalyzed by CYP7B1 in the endoplasmic reticulum membrane. Defects in CYB7B1 are associated with failure of 7alpha-hydroxylation in vivo, and with liver damage, confirming both the function of the enzyme and the central role of the liver in this metabolic process (Setchell et al. 1998).
REACT_9998 (Reactome) The isomerization of 25(R) THCA-CoA to 25(S) THCA-CoA, catalyzed by 2-methylacyl-CoA racemase, occurs in the peroxisomal matrix.
SCP2-1REACT_10103 (Reactome)
SCP2-1REACT_10113 (Reactome)
SLC10A1REACT_9958 (Reactome)
SLC10A2REACT_10050 (Reactome)
SLC27A2REACT_10049 (Reactome)
SLC27A2REACT_10106 (Reactome)
SLC27A2REACT_10119 (Reactome)
SLC27A2REACT_9970 (Reactome)
SLC27A5REACT_10000 (Reactome)
SLC27A5REACT_10020 (Reactome)
SLC27A5REACT_10034 (Reactome)
SLC27A5REACT_10064 (Reactome)
SLC27A5REACT_10087 (Reactome)
SLCO1A2REACT_10072 (Reactome)
SLCO1B1REACT_10091 (Reactome)
SLCO1B3REACT_10124 (Reactome)
THCA-CoAArrowREACT_10074 (Reactome)
THCA-CoAREACT_10019 (Reactome)
THCAArrowREACT_10001 (Reactome)
THCAREACT_10020 (Reactome)
THCAREACT_10049 (Reactome)
TetraHCAArrowREACT_10100 (Reactome)
TetraHCAREACT_10000 (Reactome)
TetraHCAREACT_10106 (Reactome)
albumin bile salt complexREACT_9958 (Reactome)
bile salts and acids ArrowREACT_10072 (Reactome)
bile salts and acids complexed with FABP6ArrowREACT_10050 (Reactome)
bile salts and acids complexed with FABP6REACT_10078 (Reactome)
bile salts and acids complexed with albuminArrowREACT_10078 (Reactome)
bile salts and acidsREACT_10050 (Reactome)
bile saltsArrowREACT_10101 (Reactome)
bile saltsArrowREACT_9958 (Reactome)
bile saltsArrowREACT_9991 (Reactome)
bile saltsREACT_10101 (Reactome)
chenodeoxycholate bile saltsArrowREACT_9968 (Reactome)
chenodeoxycholoyl-CoAArrowREACT_10103 (Reactome)
chenodeoxycholoyl-CoAREACT_9968 (Reactome)
cholate bile saltsArrowREACT_10091 (Reactome)
cholate bile saltsArrowREACT_10124 (Reactome)
cholate bile saltsArrowREACT_9974 (Reactome)
cholate; chenodeoxycholateREACT_10034 (Reactome)
cholateArrowREACT_10061 (Reactome)
choloyl-CoA; chenodeoxycholoyl-CoAArrowREACT_10034 (Reactome)
choloyl-CoA; chenodeoxycholoyl-CoAREACT_9991 (Reactome)
choloyl-CoAArrowREACT_10113 (Reactome)
choloyl-CoAREACT_10061 (Reactome)
choloyl-CoAREACT_9974 (Reactome)
glycine; taurineREACT_9968 (Reactome)
glycine; taurineREACT_9974 (Reactome)
glycine; taurineREACT_9991 (Reactome)
propionyl CoAArrowREACT_10103 (Reactome)
propionyl CoAArrowREACT_10113 (Reactome)
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