ACOT8
ACTEIII
PTE1
PTE2
UniProt:O14734 ACOT8
ACSVL1
FACVL1
FATP2
SLC27A2
UniProt:O14975 SLC27A2
VLACS
ABCC3
CMOAT2
MLP2
MRP3
UniProt:O15438 ABCC3
ABCB11
BSEP
UniProt:O95342 ABCB11
SCP2
UniProt:P22307-1 SCP2
OATP
OATP1
OATP1A2
SLC21A3
SLCO1A2
UniProt:P46721 SLCO1A2
AKR1D1
SRD5B1
UniProt:P51857 AKR1D1
CYP27
CYP27A1
UniProt:Q02318 CYP27A1
ASBT
ISBT
NTCP2
SLC10A2
UniProt:Q12908 SLC10A2
BAAT
UniProt:Q14032 BAAT
GIG29
NTCP
SLC10A1
UniProt:Q14973 SLC10A1
HSD3B7
UniProt:Q9H2F3 HSD3B7
LST2
OATP1B3
OATP8,SLC21A8
SLCO1B3
UniProt:Q9NPD5 SLCO1B3
CYP39A1
UniProt:Q9NYL5 CYP39A1
AMACR
UniProt:Q9UHK6 AMACR
ACSB
ACSVL6
FACVL3
FATP5
SLC27A5
UniProt:Q9Y2P5 SLC27A5
CYP46
CYP46A1
UniProt:Q9Y6A2 CYP46A1
LST1
OATP1B1
OATP2
OATPC
SLC21A6
SLCO1B1
UniProt:Q9Y6L6 SLCO1B1
Transport (influx) of bile salts and acids by OATP-A
Transport (influx) of glycocholate and taurocholate by OATP-8
Transport (influx) of glycocholate and taurocholate by OATP-C
Cholesterol + NADPH + H+ + O2 => 7-alpha-hydroxycholesterol + NADP+ + H2O
Cholesterol is hydroxylated to 7alpha-hydroxycholesterol by CYP7A1
7alpha-hydroxycholesterol is oxidized and isomerized to 4-cholesten-7alpha-ol-3-one
4-Cholesten-7alpha-ol-3-one is 12alpha-hydroxylated to 7-alpha,12-alpha-dihydroxycholest-4-en-3-one
7-alpha-hydroxycholest-4-en-3-one + NADPH + H+ + O2 => 7-alpha,12-alpha-dihydroxycholest-4-en-3-one + NADP+ + H2O
4-cholesten-7alpha, 12alpha-diol-3-one + NADPH + H+ => 5beta-cholesten-7alpha,12alpha-diol-3-one + NADP+
4-cholesten-7alpha, 12alpha-diol-3-one is reduced to 5beta-cholesten-7alpha, 12alpha-diol-3-one
4-cholesten-7alpha-ol-3-one + NADPH + H+ => 5beta-cholestan-7alpha-ol-3-one + NADP+
4-cholesten-7alpha-ol-3-one is reduced to 5beta-cholestan-7alpha-ol-3-one
5Beta-cholesten-7alpha, 12alpha-diol-3-one + NADPH + H+ => 5beta-cholestan-3alpha, 7alpha, 12alpha-triol + NAPDP+
5Beta-cholesten-7alpha, 12alpha-diol-3-one is reduced to 5beta-cholestan-3alpha, 7alpha, 12alpha-triol
5beta-cholestan-7alpha-ol-3-one is reduced to 5beta-cholestan-3alpha, 7alpha-diol
5beta-cholestan-7alpha-ol-3-one+ NADPH + H+ => 5beta-cholestan-3alpha, 7alpha-diol + NADP+
5beta-cholestan-3alpha, 7alpha, 12alpha-triol is translocated from the cytosol to the mitochondrial matrix
5beta-cholestan-3alpha, 7alpha-diol is translocated from the cytosol to the mitochondrial matrix
5beta-cholestan-3alpha, 7alpha, 12alpha-triol + NADPH + H+ + O2 => 5beta-cholestan-3alpha, 7alpha, 12alpha, 27-tetrol + NADP+ + H2O
5beta-cholestan-3alpha, 7alpha, 12alpha-triol is hydroxylated to 5beta-cholestan-3alpha, 7alpha, 12alpha, 27-tetrol
5beta-cholestan-3alpha, 7alpha-diol + NADPH + H+ + O2 => 5beta-cholestan-3alpha, 7alpha, 26-triol + NADP+ + H2O
5beta-cholestan-3alpha, 7alpha-diol is hydroxylated to 5beta-cholestan-3alpha, 7alpha, 26-triol
5beta-cholestan-3alpha,7alpha,12alpha,27-tetrol + NADP+ => 3alpha,7alpha,12alpha-trihydroxy-5beta-cholestan-27-al + NADPH + H+
5beta-cholestan-3alpha,7alpha,12alpha,27-tetrol is oxidized to 3alpha,7alpha,12alpha-trihydroxy-5beta-cholestan-27-al
5beta-cholestan-3alpha, 7alpha, 26-triol + NADP+ => 3alpha,7alpha-dihydroxy-5beta-cholestan-26-al + NADPH + H+
5beta-cholestan-3alpha, 7alpha, 26-triol is oxidized to 3alpha, 7alpha-dihydroxy-5beta-cholestan-26-al
3alpha,7alpha,12alpha-trihydroxy-5beta-cholestan-27-al + NADPH + H+ + O2 => 3alpha,7alpha,12alpha-trihydroxy-5beta-cholestanoate (THCA) + NADP+ + H2O
3alpha,7alpha,12alpha-trihydroxy-5beta-cholestan-27-al is oxidized to 3alpha,7alpha,12alpha-trihydroxy-5beta-cholestanoate (THCA)
3alpha, 7alpha-dihydroxy-5beta-cholestan-26-al + NADPH + H+ + O2 => 3alpha, 7alpha-dihydroxy-5beta-cholestanoate (DHCA) + NADP+ + H2O
3alpha, 7alpha-dihydroxy-5beta-cholestan-26-al is oxidized to 3alpha, 7alpha-dihydroxy-5beta-cholestanoate (DHCA)
THCA [mitochondrial matrix] => THCA [cytosol]
THCA is translocated from the mitochondrial matrix to the cytosol
DHCA [mitochondrial matrix] => DHCA [cytosol]
DHCA is translocated from the mitochondrial matrix to the cytosol
THCA + ATP + CoASH => 25(R) THCA-CoA + AMP + pyrophosphate (SLC27A2 "VLCS")
THCA is conjugated with Coenzyme A (SLC27A2 VLCS)
DHCA + ATP + CoASH => 25(R) DHCA-CoA + AMP + pyrophosphate (SLC27A2 "VLCS")
DHCA is conjugated with Coenzyme A (SLC27A2 VLCS)
THCA + ATP + CoASH => 25(R) THCA-CoA + AMP + pyrophosphate (SCLS25A5 "BACS")
THCA is conjugated with Coenzyme A (SLC27A5 BACS)
DHCA + ATP + CoASH => 25(R) DHCA-CoA + AMP + pyrophosphate (SCLS25A5 "BACS")
DHCA is conjugated with Coenzyme A (SLC27A5 BACS)
25(R) THCA-CoA [ cytosol] => 25(R) THCA-CoA [peroxisome]
25(R) THCA-CoA is translocated from the cytosol to the peroxisome
25(R) DHCA-CoA [ cytosol] => 25(R) DHCA-CoA [peroxisome]
25(R) DHCA-CoA is translocated from the cytosol to the peroxisome
25(R) THCA-CoA => 25(S) THCA-CoA
Isomerization of 25(R) THCA-CoA to 25(S) THCA-CoA
25(R) DHCA-CoA => 25(S) DHCA-CoA
Isomerization of 25(R) DHCA-CoA to 25(S) DHCA-CoA
25(S) THCA-CoA + O2 => THCA-CoA + H2O2
25(S) THCA-CoA is dehydrogenated to 3alpha,7alpha,12alpha-trihydroxy-5beta-cholest-24-enoyl-CoA (THCA-CoA)
25(S) DHCA-CoA + O2 => 25(S) 3alpha,7alpha-dihydroxy-5beta-cholest-24-enoyl-CoA + H2O2
25(S) DHCA-CoA is dehydrogenated to 25(S) 3alpha,7alpha-dihydroxy-5beta-cholest-24-enoyl-CoA
3alpha,7alpha,12alpha-trihydroxy-5beta-cholest-24-enoyl-CoA (THCA-CoA) is hydrated to (24R, 25R) 3alpha,7alpha,12alpha,24-tetrahydroxy-5beta-cholestanoyl-CoA
3alpha,7alpha,12alpha-trihydroxy-5beta-cholest-24-enoyl-CoA + H2O => (24R, 25R) 3alpha,7alpha,12alpha,24-tetrahydroxy-5beta-cholestanoyl-CoA
25(S) 3alpha,7alpha-dihydroxy-5beta-cholest-24-enoyl-CoA + H2O => (24R, 25R) 3alpha,7alpha,24-trihydroxy-5beta-cholestanoyl-CoA
25(S) 3alpha,7alpha-dihydroxy-5beta-cholest-24-enoyl-CoA is hydrated to (24R, 25R) 3alpha,7alpha,24-trihydroxy-5beta-cholestanoyl-CoA
(24R, 25R) 3alpha,7alpha,12alpha,24-tetrahydroxy-5beta-cholestanoyl-CoA + NAD+ => 3alpha,7alpha,12alpha-trihydroxy-5beta-cholest-24-one-CoA + NADH + H+
(24R, 25R) 3alpha,7alpha,12alpha,24-tetrahydroxy-5beta-cholestanoyl-CoA is oxidized to 3alpha,7alpha,12alpha-trihydroxy-5beta-cholest-24-one-CoA
(24R, 25R) 3alpha,7alpha,24-trihydroxy-5beta-cholestanoyl-CoA + NAD+ => 3alpha,7alpha-dihydroxy-5beta-cholest-24-one-CoA + NADH + H+
(24R, 25R) 3alpha,7alpha,24-trihydroxy-5beta-cholestanoyl-CoA is oxidized to 3alpha,7alpha-dihydroxy-5beta-cholest-24-one-CoA
3alpha,7alpha,12alpha-trihydroxy-5beta-cholan-24-one-CoA + CoASH => choloyl-CoA (3alpha,7alpha,12alpha-trihydroxy-5beta-cholan-24-one-CoA) + propionyl CoA
Thiolysis of 3alpha,7alpha,12alpha-trihydroxy-5beta-cholan-24-one-CoA yields choloyl-CoA (3alpha,7alpha,12alpha-trihydroxy-5beta-cholan-24-one-CoA) and propionyl CoA
3alpha,7alpha-dihydroxy-5beta-cholan-24-one-CoA + CoASH => chenodeoxycholoyl-CoA (3alpha,7alpha-dihydroxy-5beta-cholan-24-one-CoA) + propionyl CoA
Thiolysis of 3alpha,7alpha-dihydroxy-5beta-cholan-24-one-CoA yields chenodeoxycholoyl-CoA (3alpha,7alpha-dihydroxy-5beta-cholan-24-one-CoA) and propionyl CoA
Hydrolysis of choloyl-CoA to cholate and CoASH
Cholate is translocated from the peroxisomal matrix to the cytosol
cholate [peroxisomal matrix] => cholate [cytosol]
Choloyl CoA reacts with glycine or taurine to form glycocholate or taurocholate
Chenodeoxycholoyl CoA reacts with glycine or taurine to form glycochenodeoxycholate or taurochenodeoxycholate
Bile salts are translocated from the peroxisomal matrix to the cytosol
bile salts [peroxisomal matrix] => cholate bile salts [cytosol]
Transport (efflux) of bile salts by ABCB11 (bile salt export pump)
Cholesterol is hydroxylated to 24-hydroxycholesterol by CYP46A1
cholesterol + NADPH + H+ + O2 => 24-hydroxycholesterol + NADP+ + H2O
Efflux of 24-hydroxycholesterol
Influx of 24-hydroxycholesterol
24-hydroxycholesterol + NADPH + H+ + O2 => cholest-5-ene-3beta,7alpha,24-triol + NADP+ + H2O
24-hydroxycholesterol is 7alpha-hydroxylated to yield cholest-5-ene-3beta,7alpha,24-triol
Cholest-5-ene-3beta,7alpha,24(S)-triol + NAD+ => 4-cholesten-7alpha,24(S)-diol-3-one + NADH + H+
Cholest-5-ene-3beta,7alpha,24(S)-triol is oxidized and isomerized to 4-cholesten-7alpha,24(S)-diol-3-one
4-Cholesten-7alpha,24(S)-diol-3-one + NADPH + H+ + O2 => 4-cholesten-7-alpha,12-alpha,24(S)-triol-3-one + NADP+ + H2O
4-Cholesten-7alpha,24(S)-diol-3-one is 12alpha-hydroxylated to 4-cholesten-7-alpha,12-alpha,24(S)-triol-3-one
4-cholesten-7alpha,12alpha,24(S)-triol-3-one + NADPH + H+ => 5beta-cholestan-7alpha,12alpha,24(S)-triol-3-one + NADP+
4-cholesten-7alpha,12alpha,24(S)-triol-3-one is reduced to 5beta-cholestan-7alpha,12alpha,24(S)-triol-3-one
4-cholesten-7alpha,24(S)-diol-3-one + NADPH + H+ => 5beta-cholestan-7alpha,24(S)-diol-3-one + NADP+
4-cholesten-7alpha,24(S)-diol-3-one is reduced to 5beta-cholestan-7alpha,24(S)-diol-3-one
5Beta-cholestan-7alpha,12alpha,24(S)-triol-3-one + NADPH + H+ => 5beta-cholestan-3alpha,7alpha,12alpha,24(S)-tetrol + NAPDP+
5Beta-cholestan-7alpha,12alpha,24(S)-triol-3-one is reduced to 5beta-cholestan-3alpha,7alpha,12alpha,24(S)-tetrol
5beta-cholestan-7alpha,24(S)-diol-3-one is reduced to 5beta-cholestan-3alpha,7alpha,24(S)-triol
5beta-cholestan-7alpha,24(S)-diol-3-one+ NADPH + H+ => 5beta-cholestan-3alpha,7alpha,24(S)-triol + NADP+
5beta-cholestan-3alpha,7alpha,12alpha,24(S)-tetrol is translocated from the cytosol to the mitochondrial matrix
5beta-cholestan-3alpha,7alpha,24(S)-triol is translocated from the cytosol to the mitochondrial matrix
5beta-cholestan-3alpha,7alpha,12alpha,24(S)-tetrol + NADPH + H+ + O2 => 5beta-cholestan-3alpha,7alpha,12alpha,24(S),27-pentol + NADP+ + H2O
5beta-cholestan-3alpha,7alpha,12alpha,24(S)-tetrol is hydroxylated to 5beta-cholestan-3alpha,7alpha,12alpha,24(S), 27-pentol
5beta-cholestan-3alpha,7alpha,24(S)-triol + NADPH + H+ + O2 => 5beta-cholestan-3alpha,7alpha,24(S),27-tetrol + NADP+ + H2O
5beta-cholestan-3alpha,7alpha,24(S)-triol is hydroxylated to 5beta-cholestan-3alpha,7alpha,24(S), 27-tetrol
5beta-cholestan-3alpha,7alpha,12alpha,24(S),27-pentol + NADPH + H+ + O2 => 3alpha,7alpha,12alpha,24(S)-tetrahydroxy-5beta-cholestan-27-al + NADP+ + H2O
5beta-cholestan-3alpha,7alpha,12alpha,24(S),27-pentol is oxidized to 3alpha,7alpha,12alpha,24(S)-tetrahydroxy-5beta-cholestan-27-al
5beta-cholestan-3alpha,7alpha,24(S),27-tetrol + NADPH + H+ + O2 => 3alpha,7alpha,24(S)-trihydroxy-5beta-cholestan-27-al + NADP+ + H2O
5beta-cholestan-3alpha,7alpha,24(S),27-tetrol is oxidized to 3alpha,7alpha,24(S)-trihydroxy-5beta-cholestan-27-al
3alpha,7alpha,12alpha,24(S)-tetrahydroxy-5beta-cholestan-27-al + NADPH + H+ + O2 => 3alpha,7alpha,12alpha,24(S)-tetrahydroxy-5beta-cholestanoate (TetraHCA) + NADP+ + H2O
3alpha,7alpha,12alpha,24(S)-tetrahydroxy-5beta-cholestan-27-al is oxidized to 3alpha,7alpha,12alpha,24(S)-tetrahydroxy-5beta-cholestanoate (TetraHCA)
3alpha,7alpha,24(S)-trihydroxy-5beta-cholestan-27-al + NADPH + H+ + O2 => 3alpha,7alpha,24(S)-trihydroxy-5beta-cholestanoate (3,7,24THCA) + NADP+ + H2O
3alpha,7alpha,24(S)-trihydroxy-5beta-cholestan-27-al is oxidized to 3alpha,7alpha,24(S)-trihydroxy-5beta-cholestanoate (3,7,24THCA)
TetraHCA [mitochondrial matrix] => TetraHCA [cytosol]
TetraHCA is translocated from the mitochondrial matrix to the cytosol
3,7,24THCA [mitochondrial matrix] => 3,7,24THCA [cytosol]
3,7,24THCA is translocated from the mitochondrial matrix to the cytosol
TetraHCA + ATP + CoASH => 25(R) TetraHCA-CoA + AMP + pyrophosphate + H2O (SCLS25A5 "BACS")
TetraHCA is conjugated with Coenzyme A (SLC27A5 BACS)
3,7,24THCA + ATP + CoASH => 3,7,24THCA-CoA + AMP + pyrophosphate + H2O (SCLS25A5 "BACS")
3,7,24THCA is conjugated with Coenzyme A (SLC27A5 BACS)
TetraHCA + ATP + CoASH => 25(R) THCA-CoA + AMP + pyrophosphate + H2O (SLC27A2 "VLCS")
TetraHCA is conjugated with Coenzyme A (SLC27A2 VLCS)
3,7,24THCA + ATP + CoASH => 3,7,24THCA-CoA + AMP + pyrophosphate + H2O (SLC27A2 "VLCS")
3,7,24THCA is conjugated with Coenzyme A (SLC27A2 VLCS)
25(R) TetraHCA-CoA [ cytosol] => 25(R) TetraHCA-CoA [peroxisome]
25(R) TetraHCA-CoA is translocated from the cytosol to the peroxisome
3,7,24THCA-CoA [ cytosol] => 3,7,24THCA-CoA [peroxisome]
3,7,24THCA-CoA is translocated from the cytosol to the peroxisome
25(R) TetraHCA-CoA => (24R, 25R) 3alpha,7alpha,12alpha,24-tetrahydroxy-5beta-cholestanoyl-CoA
Isomerization of 25(R) TetraHCA-CoA to (24R, 25R) 3alpha,7alpha,12alpha,24-tetrahydroxy-5beta-cholestanoyl-CoA
3,7,24THCA-CoA => (24R, 25R) 3alpha,7alpha,24-trihydroxy-5beta-cholestanoyl-CoA
Isomerization of 3,7,24THCA-CoA to (24R, 25R) 3alpha,7alpha,24-trihydroxy-5beta-cholestanoyl-CoA
Cholesterol is hydroxylated to 27-hydroxycholesterol by CYP27
cholesterol + NADPH + H+ + O2 => 27-hydroxycholesterol + H2O + NADP+
Efflux of 27-hydroxycholesterol
Influx of 27-hydroxycholesterol
27-hydroxycholesterol + NADPH + H+ + O2 => cholest-5-ene-3beta,7alpha,27-triol + H2O + NADP+
27-hydroxycholesterol is 7alpha-hydroxylated
Cholest-5-ene-3beta,7alpha,27-triol + NAD+ => 4-cholesten-7alpha,27-diol-3-one +NADH + H+
Cholest-5-ene-3beta,7alpha,27-triol is oxidized and isomerized to 4-cholesten-7alpha,27-diol-3-one
4-Cholesten-7alpha,27-diol-3-one + NADPH + H+ + O2 => 4-Cholesten-7alpha,12alpha,27-triol-3-one + NADP+ + H2O
4-Cholesten-7alpha,27-diol-3-one is 12alpha-hydroxylated to 4-Cholesten-7alpha,12alpha,27-triol-3-one
4-cholesten-7alpha,12alpha,27-triol-3-one + NADPH + H+ => 5beta-cholestan-7alpha,12alpha,27-triol-3-one + NADP+
4-cholesten-7alpha,12alpha,27-triol-3-one is reduced to 5beta-cholestan-7alpha,12alpha,27-triol-3-one
4-cholesten-7alpha,27-diol-3-one + NADPH + H+ => 5beta-cholestan-7alpha,27-diol-3-one + NADP+
4-cholesten-7alpha,27-diol-3-one is reduced to 5beta-cholestan-7alpha,27-diol-3-one
5Beta-cholestan-7alpha,12alpha,27-triol-3-one + NADPH + H+ => 5beta-cholestan-3alpha,7alpha,12alpha,27-tetrol + NAPDP+
5Beta-cholestan-7alpha,12alpha,27-triol-3-one is reduced to 5beta-cholestan-3alpha,7alpha,12alpha,27-tetrol
5beta-cholestan-7alpha,27-diol-3-one is reduced to 5beta-cholestan-3alpha,7alpha,27-triol
5beta-cholestan-7alpha,27-diol-3-one+ NADPH + H+ => 5beta-cholestan-3alpha,7alpha,27-triol + NADP+
5beta-cholestan-3alpha,7alpha,12alpha,27-tetrol is translocated from the cytosol to the mitochondrial matrix
5beta-cholestan-3alpha,7alpha,27-triol is translocated from the cytosol to the mitochondrial matrix
Cholesterol is hydroxylated to 25-hydroxycholesterol
25-hydroxycholesterol is 7alpha-hydroxylated by CYP7B1
Co-transport (influx) of bile salts and acids and sodium ions by ASBT
Transport (efflux) of bile salts by ABCC3 (MRP3)
Co-transport (influx) of bile salts and sodium ions by NTCP
Cytosolic cholate and chenodeoxycholate are conjugated with Coenzyme A (SLC27A5 BACS)
Cytosolic chenodeoxycholoyl-CoA or choloyl-CoA are conjugated with glycine or taurine
Bile acid and bile salt metabolism
Synthesis of bile acids and bile salts
Synthesis of bile acids and bile salts via 7alpha-hydroxycholesterol
Synthesis of bile acids and bile salts via 24-hydroxycholesterol
Synthesis of bile acids and bile salts via 27-hydroxycholesterol
Recycling of bile acids and salts