Bile acid and bile salt metabolism (Homo sapiens)

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1, 23, 27, 32317, 19, 28445, 4834, 426, 821, 45, 4845, 48915, 2216481026, 43, 4417, 19, 28121, 45, 48292, 12, 251117, 19, 28111, 407, 18, 3046, 4736117, 19, 2845, 4814, 20, 382, 12, 25117, 19, 2817, 19, 283615, 2293636495, 31411137, 391621, 45, 4820165, 3117, 19, 2845, 482, 12, 25362, 12, 2517124, 35, 4113145, 4845, 4817, 19, 2845, 4816, 894319914115, 226, 83346, 4721, 45, 48136492945, 4820endoplasmic reticulum lumencytosolperoxisomal matrixendoplasmic reticulum lumenmitochondrial matrixendoplasmic reticulum lumencytosolcytosolcytosolmitochondrial matrixendoplasmic reticulum lumenendoplasmic reticulum lumenendoplasmic reticulum lumenH+Cytochrome P450 7A1NADPH5beta-cholestan-3alpha, 7alpha-diol3-oxo-5-beta-steroid 4-dehydrogenaseHSD3B7NADP+5beta-cholesten-7alpha, 12alpha-diol-3-oneH2OH+NTCPbile salts and acids25(R) TetraHCA-CoA3alpha,7alpha,12alpha-trihydroxy-5beta-cholestan-27-alNADPHNAD+CoA-SH4-cholesten-7alpha-ol-3-one27-hydroxycholesterolTetraHCAADPH+NAD+HSD17B4 dimerNADPH24-hydroxycholesterolsodium ionH+CoA-SHH2ONADPHBile acid-CoA:amino acid N-acetyltransferaseATPCholesterol25(R) TetraHCA-CoANADP+NADPHOrthophosphate24-hydroxycholesterolH2OH+bile salts and acids complexed with FABP627-hydroxycholesterolglycine; taurineASBTSerum albuminNADHCytochrome P450 7B125(S) 3alpha,7alpha-dihydroxy-5beta-cholest-24-enoyl-CoA5beta-cholestan-7alpha-ol-3-oneATPVery long-chain acyl-CoA synthetaseCoA-SHNADPHchenodeoxycholoyl-CoANAD+OATP-A5beta-cholestan-3alpha,7alpha,12alpha,27-tetrolOxygenTHCAOxygenOxygen5beta-cholestan-3alpha,7alpha,24(S)-triolH+5beta-cholestan-3alpha,7alpha,26-triolOxygenNADP+NAD+NADP+H+cholesterolalbumin:bile salt complexNADP+H2OAMACRNADP+H+bile salts25(R) THCA-CoACytochrome P450 275beta-cholestan-3alpha,7alpha,24(S),27-tetrol7 alpha-hydroxycholesterolNADP+H+3alpha,7alpha,12alpha,24(S)-tetrahydroxy-5beta-cholestan-27-alH+NADP+NADP+Oxygen25-hydroxycholesterolOxygenNADPHNADPHCoA-SH5beta-cholestan-3alpha, 7alpha, 12alpha-triolsodium ionSLC27A5(24R, 25R) 3alpha,7alpha,12alpha,24-tetrahydroxy-5beta-cholestanoyl-CoAcholate bile salts4-cholesten-7alpha, 12alpha-diol-3-oneCytochrome P450 2725(S) DHCA-CoAcholoyl-CoA; chenodeoxycholoyl-CoAalbumin:bile salt and acid (OATP-A) complexDHCAH2Ocholate bile salts4-cholesten-7alpha,27-diol-3-oneNADPHNADP+25(R) DHCA-CoAOxygenOxygenAldo-keto reductase family 1 member C4Cytochrome p450 46A1CholesterolCholest-5-ene-3beta,7alpha,25-triol25(S) THCA-CoASLCO1B1H+ATP5beta-cholestan-7alpha,12alpha,24(S)-triol-3-onecholate; chenodeoxycholateNADP+H+H2OH+NADP+NADPHSCP25beta-cholestan-3alpha,7alpha-diol3,7,24THCA-CoANADPH3-oxo-5-beta-steroid 4-dehydrogenaseH2OH2O5beta-cholestan-7alpha,12alpha,27-triol-3-oneCytochrome P450 7B1OxygenCholest-5-ene-3beta,7alpha,27-triol25(S) 3alpha,7alpha,12alpha-trihydroxy-5beta-cholest-24-enoyl-CoA5beta-cholestan-7alpha,27-diol-3-one4-cholesten-7alpha,24(S)-diol-3-oneCoA-SHH2O2Cytochrome P450 39A1Bile acid-CoA:amino acid N-acetyltransferasecholateH+ACOX2:FADNADP+H+NADP+3,7,24THCA5beta-cholestan-7alpha,24(S)-diol-3-oneNADPHchenodeoxycholate bile saltsNADPHH2OOATP-8NADPHVery long-chain acyl-CoA synthetaseATPH2O5beta-cholestan-3alpha,7alpha,12alpha,24(S)-tetrolOrthophosphateOxygenBile salt export pumpH+HSD3B7albumin:cholate bile salt complex5beta-cholestan-3alpha,7alpha,12alpha,24(S)-tetrolcholoyl-CoAH+NADP+H2OCytochrome P450 8B1AMPDHCAABCC34-cholesten-7alpha,12alpha,27-triol-3-oneCoA-SHAldo-keto reductase family 1 member C4H2ONADPHCytochrome P450 273-oxo-5-beta-steroid 4-dehydrogenaseH2OH+H2OATP24-hydroxycholesterolTHCANADPHOxygenCoA-SHH+3alpha,7alpha,24(S)-trihydroxy-5beta-cholestan-27-alNADPHNADP+NADHCH25H (Fe2+ cofactor)bile saltssodium ionOxygenOxygenCytochrome P450 27NADP+5beta-cholestan-3alpha,7alpha,24(S)-triol5beta-cholestan-3alpha,7alpha,12alpha-triolH+(24R, 25R) 3alpha,7alpha,24-trihydroxy-5beta-cholestanoyl-CoAH+H+25(R) THCA-CoACytochrome P450 27NADPHNADP+H2OAMPsodium ion3alpha,7alpha-dihydroxy-5beta-cholest-24-one-CoAAldo-keto reductase family 1 member C4NADP+5beta-cholestan-3alpha,7alpha,12alpha,27-tetrol5beta-cholestan-3alpha,7alpha,12alpha,24(S),27-pentolH+OxygenNADPHCholest-5-ene-3beta,7alpha,24(S)-triolOxygenNADPHNAD+H2O3,7,24THCA-CoACytochrome P450 8B1H+27-hydroxycholesterolH+Cholesterolbile saltsH2OSLC27A5bile salts and acids (OATP-A)glycine; taurinebile saltsNADP+5beta-cholestan-3alpha,7alpha,26-triolACOT8NADH25(R) DHCA-CoAH2OH+NADH4-cholesten-7alpha,12alpha,24(S)-triol-3-oneOxygenpyrophosphatepyrophosphateCytochrome P450 27NADP+Cytochrome P450 8B1SLC27A5HSD3B7TetraHCAcholateNADPHAMPH2Obile salts and acids complexed with albuminNADP+3alpha,7alpha-dihydroxy-5beta-cholestan-26-al3alpha,7alpha,12alpha-trihydroxy-5beta-cholest-24-one-CoAOxygenpropionyl CoAADPNADPHH2OCytochrome P450 27pyrophosphate3,7,24THCAFABP6


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).

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  64. Kondo KH, Kai MH, Setoguchi Y, Eggertsen G, Sjöblom P, Setoguchi T, Okuda KI, Björkhem I.; ''Cloning and expression of cDNA of human delta 4-3-oxosteroid 5 beta-reductase and substrate specificity of the expressed enzyme.''; PubMed Europe PMC Scholia
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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
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81480view13:01, 21 August 2015ReactomeTeamVersion53
76958view08:24, 17 July 2014ReactomeTeamFixed remaining interactions
76663view12:03, 16 July 2014ReactomeTeamFixed remaining interactions
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44976view14:24, 6 October 2011MartijnVanIerselOntology Term : 'bile acid biosynthetic pathway' added !
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External references


View all...
NameTypeDatabase referenceComment
(24R, 25R) 3alpha,

7alpha,12alpha,24- tetrahydroxy-5beta- cholestanoyl-CoA

MetaboliteREACT_10198 (Reactome)
(24R, 25R) 3alpha,

7alpha,24-trihydroxy- 5beta-cholestanoyl- CoA

Metabolite27403 (ChEBI)

hydroxychole sterol

Metabolite34310 (ChEBI)

hydroxycholes terol

Metabolite34310 (ChEBI)
25(R) DHCA-


Metabolite15494 (ChEBI)
25(R) THCA-


Metabolite37642 (ChEBI)
25(R) TetraHCA-


Metabolite27458 (ChEBI)
25(S) 3alpha,

7alpha,12alpha- trihydroxy-5beta- cholest-24-enoyl- CoA

MetaboliteREACT_10191 (Reactome)
25(S) 3alpha,

7alpha- dihydroxy- 5beta-cholest- 24-enoyl-CoA

Metabolite27393 (ChEBI)
25(S) DHCA-


MetaboliteREACT_10853 (Reactome)
25(S) THCA-


Metabolite37643 (ChEBI)

hydroxycholes terol

Metabolite42977 (ChEBI)

hydroxychole sterol

Metabolite17703 (ChEBI)

hydroxycholes terol

Metabolite17703 (ChEBI)


MetaboliteREACT_10236 (Reactome)


MetaboliteREACT_11020 (Reactome)


Metabolite27403 (ChEBI)

steroid 4- dehydrogenase

ProteinP51857 (UniProt)

12alpha,24(S)- tetrahydroxy- 5beta-cholestan- 27-al

MetaboliteREACT_10185 (Reactome)

12alpha- trihydroxy- 5beta-cholestan- 27-al

Metabolite16466 (ChEBI)

12alpha-trihydroxy- 5beta-cholest-24- one-CoA

Metabolite27379 (ChEBI)

24(S)- trihydroxy- 5beta- cholestan-27-al

MetaboliteREACT_10571 (Reactome)

dihydroxy- 5beta- cholestan-26-al

Metabolite27428 (ChEBI)

dihydroxy-5beta- cholest-24-one- CoA

Metabolite28533 (ChEBI)

7alpha, 12alpha- diol-3-one

Metabolite28477 (ChEBI)

7alpha,12alpha, 24(S)-triol-3-one

MetaboliteREACT_10249 (Reactome)

7alpha,12alpha, 27-triol-3-one

MetaboliteREACT_10863 (Reactome)

7alpha,24(S)- diol-3-one

MetaboliteREACT_10645 (Reactome)

7alpha,27-diol- 3-one

MetaboliteREACT_10821 (Reactome)

7alpha-ol-3- one

Metabolite17899 (ChEBI)

cholestan- 3alpha, 7alpha-diol

Metabolite28047 (ChEBI)

cholestan- 3alpha, 7alpha-diol

Metabolite28047 (ChEBI)

cholestan- 3alpha, 7alpha, 12alpha-triol

Metabolite16496 (ChEBI)

cholestan- 3alpha,7alpha, 12alpha,24(S), 27-pentol

MetaboliteREACT_10916 (Reactome)

cholestan- 3alpha,7alpha, 12alpha,24(S)- tetrol

Metabolite17278 (ChEBI)

cholestan- 3alpha,7alpha, 12alpha,24(S)- tetrol

MetaboliteREACT_10319 (Reactome)

cholestan- 3alpha,7alpha, 12alpha,27- tetrol

Metabolite17278 (ChEBI)

cholestan- 3alpha,7alpha, 12alpha-triol

Metabolite16496 (ChEBI)

cholestan- 3alpha,7alpha, 24(S)-triol

MetaboliteREACT_10165 (Reactome)

cholestan- 3alpha,7alpha, 24(S)-triol

MetaboliteREACT_13314 (Reactome)

cholestan- 3alpha,7alpha, 26-triol

Metabolite28540 (ChEBI)

3alpha,7alpha, 12alpha,27-tetrol

MetaboliteREACT_10938 (Reactome)

3alpha,7alpha, 24(S),27-tetrol

MetaboliteREACT_10904 (Reactome)

3alpha,7alpha,26- triol

MetaboliteREACT_10427 (Reactome)

7alpha,12alpha, 27-triol-3-one

MetaboliteREACT_10380 (Reactome)

7alpha,12alpha,24 (S)-triol-3-one

MetaboliteREACT_10574 (Reactome)

7alpha,24(S)- diol-3-one

MetaboliteREACT_10353 (Reactome)

7alpha,27-diol-3- one

MetaboliteREACT_10201 (Reactome)


Metabolite2290 (ChEBI)

7alpha, 12alpha- diol-3-one

Metabolite2288 (ChEBI)
7 alpha-

hydroxychole sterol

Metabolite17500 (ChEBI)
ABCC3 ProteinO15438 (UniProt)
ACOT8 ProteinO14734 (UniProt)
ACOX2:FAD ComplexREACT_10603 (Reactome)
ADP Metabolite16761 (ChEBI)
AMACR ProteinQ9UHK6 (UniProt)
AMP Metabolite16027 (ChEBI)
ASBT ProteinQ12908 (UniProt)
ATP Metabolite15422 (ChEBI)

reductase family 1 member C4

ProteinP17516 (UniProt)
Bile acid-CoA:

amino acid N- acetyltransferase

ProteinQ14032 (UniProt)
Bile salt

export pump

ProteinO95342 (UniProt)
CH25H (Fe2+


ComplexREACT_10164 (Reactome)

3beta,7alpha, 25-triol

Metabolite37623 (ChEBI)

3beta,7alpha, 27-triol

Metabolite18431 (ChEBI)

3beta,7alpha,24 (S)-triol

Metabolite37640 (ChEBI)
Cholesterol Metabolite16113 (ChEBI)
CoA-SH Metabolite15346 (ChEBI)

P450 27

ProteinQ02318 (UniProt)

P450 39A1

ProteinQ9NYL5 (UniProt)

P450 7A1

ProteinP22680 (UniProt)

P450 7B1

ProteinO75881 (UniProt)

P450 8B1

ProteinQ9UNU6 (UniProt)

p450 46A1

ProteinQ9Y6A2 (UniProt)
DHCA Metabolite16577 (ChEBI)
FABP6 ProteinP51161 (UniProt)
H+ Metabolite15378 (ChEBI)
H2O Metabolite15377 (ChEBI)
H2O2 Metabolite16240 (ChEBI)


ComplexREACT_17840 (Reactome)
HSD3B7 ProteinQ9H2F3 (UniProt)
NAD+ Metabolite15846 (ChEBI)
NADH Metabolite16908 (ChEBI)
NADP+ Metabolite18009 (ChEBI)
NADPH Metabolite16474 (ChEBI)
NTCP ProteinQ14973 (UniProt)
OATP-8 ProteinQ9NPD5 (UniProt)
OATP-A ProteinP46721 (UniProt)


Metabolite18367 (ChEBI)
Oxygen Metabolite15379 (ChEBI)
SCP2 ProteinP22307-1 (UniProt)
SLC27A5 ProteinQ9Y2P5 (UniProt)
SLCO1B1 ProteinQ9Y6L6 (UniProt)


ProteinP02768 (UniProt)
THCA Metabolite18402 (ChEBI)
TetraHCA MetaboliteREACT_10686 (Reactome)
TetraHCA MetaboliteREACT_10721 (Reactome)
Very long-

chain acyl-CoA synthetase

ProteinO14975 (UniProt)
albumin:bile salt

and acid (OATP-A) complex

ComplexREACT_10829 (Reactome)
albumin:bile salt


ComplexREACT_10145 (Reactome)

bile salt complex

ComplexREACT_10265 (Reactome)
bile salts MetaboliteREACT_10276 (Reactome)
bile salts MetaboliteREACT_10834 (Reactome)
bile salts MetaboliteREACT_11011 (Reactome)
bile salts and


MetaboliteREACT_9533 (Reactome)
bile salts and

acids (OATP-A)

MetaboliteREACT_10444 (Reactome)
bile salts and

acids complexed with FABP6

ComplexREACT_10401 (Reactome)
bile salts and

acids complexed with albumin

ComplexREACT_10309 (Reactome)

holate bile salts

MetaboliteREACT_10485 (Reactome)


Metabolite28701 (ChEBI)
cholate Metabolite16359 (ChEBI)
cholate bile


MetaboliteREACT_10914 (Reactome)
cholate bile salts MetaboliteREACT_10246 (Reactome)


MetaboliteREACT_10869 (Reactome)
cholesterol Metabolite16113 (ChEBI)


Metabolite15519 (ChEBI)

chenodeoxycholoyl- CoA

MetaboliteREACT_10852 (Reactome)


MetaboliteREACT_10219 (Reactome)


MetaboliteREACT_10925 (Reactome)
propionyl CoA Metabolite15539 (ChEBI)


Metabolite29888 (ChEBI)


Metabolite29101 (ChEBI)

Annotated Interactions

No annotated interactions

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