Bile acid and bile salt metabolism

Pathway network for the Bile acid and bile salt metabolism SuperPath

Sources:

Reactome
WikiPathways

Pathways in the Bile acid and bile salt metabolism SuperPath

#	Name	Source	Genes
1	Bile acid and bile salt metabolism	Reactome	ABCB11 ABCC3 ACOT8 ACOX2 AKR1C1 AKR1C2 AKR1C3 AKR1C4 AKR1D1 ALB AMACR BAAT CH25H CYP27A1 CYP39A1 CYP46A1 CYP7A1 CYP7B1 CYP8B1 FABP6 HSD17B4 HSD3B7 NCOA1 NCOA2 NR1H4 OSBP OSBPL1A OSBPL2 OSBPL3 OSBPL6 OSBPL7 OSBPL9 RXRA SCP2 SLC10A1 SLC10A2 SLC27A2 SLC27A5 SLC51A SLC51B SLCO1A2 SLCO1B1 SLCO1B3 STARD5 (see all 44) (see less)
2	Synthesis of bile acids and bile salts	Reactome	ABCB11 ACOT8 ACOX2 AKR1C1 AKR1C2 AKR1C3 AKR1C4 AKR1D1 AMACR BAAT CH25H CYP27A1 CYP39A1 CYP46A1 CYP7A1 CYP7B1 CYP8B1 HSD17B4 HSD3B7 NCOA1 NCOA2 NR1H4 OSBP OSBPL1A OSBPL2 OSBPL3 OSBPL6 OSBPL7 OSBPL9 RXRA SCP2 SLC27A2 SLC27A5 (see all 33) (see less)
3	Disorders of bile acid synthesis and biliary transport	WikiPathways	ABCB1 ABCB11 ABCB4 ABCC2 ABCC3 ABCC4 ABCD3 ACOX2 AKR1C4 AKR1D1 ATP8B1 BAAT CYP27A1 CYP7A1 CYP7B1 FABP6 HSD17B4 HSD3B7 SLC10A1 SLC10A2 SLC27A2 SLC27A5 SLC51A SLC51B SLCO1B1 SLCO1B3 (see all 26) (see less)
4	Synthesis of bile acids and bile salts via 7alpha-hydroxycholesterol	Reactome	ABCB11 ACOT8 ACOX2 AKR1C1 AKR1C2 AKR1C3 AKR1C4 AKR1D1 AMACR BAAT CYP27A1 CYP7A1 CYP7B1 CYP8B1 HSD17B4 HSD3B7 NCOA1 NCOA2 NR1H4 RXRA SCP2 SLC27A2 SLC27A5 (see all 23) (see less)
5	Recycling of bile acids and salts	Reactome	ABCB11 ABCC3 ALB BAAT FABP6 NCOA1 NCOA2 NR1H4 RXRA SLC10A1 SLC10A2 SLC27A5 SLC51A SLC51B SLCO1A2 SLCO1B1 SLCO1B3 STARD5 (see all 18) (see less)
6	Drug induction of bile acid pathway	WikiPathways	ABCB1 ABCB11 ABCC2 ABCC3 ABCC4 BAAT CYP3A4 CYP7A1 NR1H4 NR1I2 NR1I3 SLC10A1 SLC51A SLC51B SLCO1B1 SULT2A1 VDR (see all 17) (see less)
7	Synthesis of bile acids and bile salts via 27-hydroxycholesterol	Reactome	AKR1C1 AKR1C2 AKR1C3 AKR1C4 AKR1D1 CYP27A1 CYP7A1 CYP7B1 CYP8B1 HSD3B7 NCOA1 NCOA2 NR1H4 RXRA (see all 14) (see less)
8	Synthesis of bile acids and bile salts via 24-hydroxycholesterol	Reactome	AKR1C1 AKR1C2 AKR1C3 AKR1C4 AKR1D1 AMACR CYP27A1 CYP39A1 CYP46A1 CYP8B1 HSD3B7 SLC27A2 SLC27A5 (see all 13) (see less)
9	Enterohepatic circulation of bile acids	WikiPathways	ABCB1 ABCB11 ABCC2 ABCC3 ABCC4 FABP6 SLC10A1 SLC10A2 SLC51A SLC51B SLCO1B1 SLCO1B3 (see all 12) (see less)
10	Defective CYP7B1 causes SPG5A and CBAS3	Reactome	CYP7B1

Gene overlap in member pathways for Bile acid and bile salt metabolism SuperPath

Ordered by rank within the SuperPath, via the multiplicity of each gene in the constituent pathways

#	Symbol	Description	Category	# Related Pathways
1	ABCB11	ATP Binding Cassette Subfamily B Member 11	Protein Coding	7
2	SLC27A5	Solute Carrier Family 27 Member 5	Protein Coding	6
3	AKR1C4	Aldo-Keto Reductase Family 1 Member C4	Protein Coding	6
4	CYP7A1	Cytochrome P450 Family 7 Subfamily A Member 1	Protein Coding	6
5	CYP27A1	Cytochrome P450 Family 27 Subfamily A Member 1	Protein Coding	6
6	BAAT	Bile Acid-CoA:Amino Acid N-Acyltransferase	Protein Coding	6
7	AKR1D1	Aldo-Keto Reductase Family 1 Member D1	Protein Coding	6
8	HSD3B7	Hydroxy-Delta-5-Steroid Dehydrogenase, 3 Beta- And Steroid Delta-Isomerase 7	Protein Coding	6
9	CYP7B1	Cytochrome P450 Family 7 Subfamily B Member 1	Protein Coding	6
10	NR1H4	Nuclear Receptor Subfamily 1 Group H Member 4	Protein Coding	6
11	NCOA2	Nuclear Receptor Coactivator 2	Protein Coding	5
12	SLCO1B1	Solute Carrier Organic Anion Transporter Family Member 1B1	Protein Coding	5
13	SLC27A2	Solute Carrier Family 27 Member 2	Protein Coding	5
14	SLC51B	SLC51 Subunit Beta	Protein Coding	5
15	CYP8B1	Cytochrome P450 Family 8 Subfamily B Member 1	Protein Coding	5
16	AKR1C1	Aldo-Keto Reductase Family 1 Member C1	Protein Coding	5
17	AKR1C2	Aldo-Keto Reductase Family 1 Member C2	Protein Coding	5
18	SLC51A	Solute Carrier Family 51 Member A	Protein Coding	5
19	RXRA	Retinoid X Receptor Alpha	Protein Coding	5
20	SLC10A1	Solute Carrier Family 10 Member 1	Protein Coding	5
21	AKR1C3	Aldo-Keto Reductase Family 1 Member C3	Protein Coding	5
22	NCOA1	Nuclear Receptor Coactivator 1	Protein Coding	5
23	ABCC3	ATP Binding Cassette Subfamily C Member 3	Protein Coding	5
24	FABP6	Fatty Acid Binding Protein 6	Protein Coding	4
25	AMACR	Alpha-Methylacyl-CoA Racemase	Protein Coding	4
26	SLCO1B3	Solute Carrier Organic Anion Transporter Family Member 1B3	Protein Coding	4
27	HSD17B4	Hydroxysteroid 17-Beta Dehydrogenase 4	Protein Coding	4
28	SLC10A2	Solute Carrier Family 10 Member 2	Protein Coding	4
29	ACOX2	Acyl-CoA Oxidase 2	Protein Coding	4
30	ACOT8	Acyl-CoA Thioesterase 8	Protein Coding	3
31	CYP46A1	Cytochrome P450 Family 46 Subfamily A Member 1	Protein Coding	3
32	CYP39A1	Cytochrome P450 Family 39 Subfamily A Member 1	Protein Coding	3
33	SCP2	Sterol Carrier Protein 2	Protein Coding	3
34	ABCB1	ATP Binding Cassette Subfamily B Member 1	Protein Coding	3
35	ABCC4	ATP Binding Cassette Subfamily C Member 4 (PEL Blood Group)	Protein Coding	3
36	ABCC2	ATP Binding Cassette Subfamily C Member 2	Protein Coding	3
37	OSBPL1A	Oxysterol Binding Protein Like 1A	Protein Coding	2
38	OSBPL6	Oxysterol Binding Protein Like 6	Protein Coding	2
39	OSBPL7	Oxysterol Binding Protein Like 7	Protein Coding	2
40	OSBPL9	Oxysterol Binding Protein Like 9	Protein Coding	2
41	ALB	Albumin	Protein Coding	2
42	OSBPL3	Oxysterol Binding Protein Like 3	Protein Coding	2
43	OSBP	Oxysterol Binding Protein~Name Same As:- HGNC:17242	Protein Coding	2
44	SLCO1A2	Solute Carrier Organic Anion Transporter Family Member 1A2	Protein Coding	2
45	STARD5	StAR Related Lipid Transfer Domain Containing 5	Protein Coding	2
46	CH25H	Cholesterol 25-Hydroxylase	Protein Coding	2
47	OSBPL2	Oxysterol Binding Protein Like 2	Protein Coding	2
48	ABCD3	ATP Binding Cassette Subfamily D Member 3	Protein Coding	1
49	ABCB4	ATP Binding Cassette Subfamily B Member 4	Protein Coding	1
50	ATP8B1	ATPase Phospholipid Transporting 8B1	Protein Coding	1
51	NR1I3	Nuclear Receptor Subfamily 1 Group I Member 3	Protein Coding	1
52	NR1I2	Nuclear Receptor Subfamily 1 Group I Member 2	Protein Coding	1
53	VDR	Vitamin D Receptor	Protein Coding	1
54	SULT2A1	Sulfotransferase Family 2A Member 1	Protein Coding	1
55	CYP3A4	Cytochrome P450 Family 3 Subfamily A Member 4	Protein Coding	1

Disorders associated with Bile acid and bile salt metabolism SuperPath

according to GeneCards Suite gene sharing

#	Disorder	Type	Genes	Score
1	Hereditary spastic paraplegia	Direct
2	Intrahepatic cholestasis of pregnancy	Enrichment	ABCB11, ABCB4, ATP8B1	7.60
3	Progressive familial intrahepatic cholestasis	Enrichment	ABCB11, ABCB4, ATP8B1	6.66
4	Congenital bile acid synthesis defect	Enrichment	AKR1D1, HSD3B7	6.07
5	Hyperbilirubinemia, rotor type	Enrichment	SLCO1B1, SLCO1B3	5.67
6	46,xy sex reversal 8	Enrichment	AKR1C2, AKR1C4	5.59
7	Cholestasis, progressive familial intrahepatic, 3	Enrichment	ABCB11, ABCB4	5.45
8	Cholestasis, intrahepatic, of pregnancy 3	Enrichment	ABCB11, ABCB4	5.45
9	Intrahepatic cholestasis	Enrichment	ABCB11, ABCB4	5.45
10	Familial hypercholanemia	Enrichment	BAAT, SLC10A1	4.66
11	Cholestasis, progressive familial intrahepatic, 1	Enrichment	ABCB4, ATP8B1	4.28
12	Colchicine resistance	Enrichment	ABCB1	3.05
13	Encephalopathy, acute transient	Enrichment	ABCB1	3.05
14	Bile acid malabsorption, primary, 1	Enrichment	SLC10A2	3.05
15	Hypercholanemia, familial, 2	Enrichment	SLC10A1	3.05
16	Inflammatory bowel disease 13	Enrichment	ABCB1	3.05
17	Cholestasis, progressive familial intrahepatic, 6	Enrichment	SLC51A	3.05
18	Bile acid synthesis defect, congenital, 2	Enrichment	AKR1D1	3.02
19	Cerebrotendinous xanthomatosis	Enrichment	CYP27A1	3.02
20	Bile acid synthesis defect, congenital, 1	Enrichment	HSD3B7	3.02
21	Bile acid synthesis defect, congenital, 3	Enrichment	CYP7B1	2.99
22	Cholestasis, progressive familial intrahepatic, 5	Enrichment	NR1H4	2.99
23	Hypercholesterolemia due to cholesterol 7alpha-hydroxylase deficiency	Enrichment	CYP7A1	2.99
24	Vitamin d-dependent rickets, type 3	Enrichment	CYP3A4	2.90
25	Hypercholanemia, familial 3	Enrichment	BAAT	2.90
26	Rickets	Enrichment	VDR	2.90
27	Hyperthyroxinemia, familial dysalbuminemic	Enrichment	ALB	2.88
28	Congenital analbuminemia	Enrichment	ALB	2.88
29	Analbuminemia	Enrichment	ALB	2.88
30	Bile acid synthesis defect, congenital, 6	Enrichment	ACOX2	2.77
31	Leukoencephalopathy with dystonia and motor neuropathy	Enrichment	SCP2	2.77
32	Cholestasis, benign recurrent intrahepatic, 2	Enrichment	ABCB11	2.75
33	Cholestasis, progressive familial intrahepatic, 4	Enrichment	ABCB11	2.75
34	Cholestasis, progressive familial intrahepatic, 2	Enrichment	ABCB11	2.75
35	Bile acid malabsorption, primary, 2	Enrichment	SLC51B	2.75
36	Bile acid synthesis defect, congenital, 4	Enrichment	AMACR	2.72
37	Alpha-methylacyl-coa racemase deficiency	Enrichment	AMACR	2.72
38	Cic-rearranged sarcoma	Enrichment	AKR1C2	2.72
39	Gallbladder disease 1	Enrichment	ABCB4	2.72
40	Bile acid synthesis defect, congenital, 5	Enrichment	ABCD3	2.72
41	Spastic paraplegia 5a, autosomal recessive	Enrichment	CYP7B1	2.69
42	Deafness, autosomal dominant 67	Enrichment	OSBPL2	2.61
43	Dubin-johnson syndrome	Enrichment	ABCC2	2.58
44	Cholestasis	Enrichment	SLC51B	2.58
45	D-bifunctional protein deficiency	Enrichment	HSD17B4	2.29
46	Pseudoxanthoma elasticum	Enrichment	ABCC2	2.28
47	Diarrhea	Enrichment	SLC51B	2.28
48	Ehlers-danlos syndrome, arthrochalasia type, 1	Enrichment	ALB	2.28
49	Developmental dysplasia of the hip 1	Enrichment	AKR1C1	2.24
50	Cholestasis, intrahepatic, of pregnancy, 1	Enrichment	ATP8B1	2.24
51	Cholestasis, benign recurrent intrahepatic, 1	Enrichment	ATP8B1	2.24
52	Vitamin d-dependent rickets, type 2a	Enrichment	VDR	2.20
53	Gilbert syndrome	Enrichment	SLCO1B1	2.01
54	Epilepsy, idiopathic generalized	Enrichment	ABCB1	2.01
55	Pulmonary disease, chronic obstructive	Enrichment	CYP46A1	1.91
56	Oculopharyngodistal myopathy 1	Enrichment	ABCD3	1.87
57	Perrault syndrome	Enrichment	HSD17B4	1.87
58	Perrault syndrome 2	Enrichment	HSD17B4	1.82
59	Perrault syndrome 1	Enrichment	HSD17B4	1.57
60	Hepatocellular carcinoma	Enrichment	VDR	1.56
61	Spastic ataxia	Enrichment	CYP7B1	1.27
62	Myeloma, multiple	Enrichment	RXRA	1.25
63	Rare autosomal dominant non-syndromic sensorineural deafness type dfna	Enrichment	OSBPL2	0.92
64	Rare genetic deafness	Enrichment	HSD17B4	0.90
65	Congenital nervous system abnormality	Enrichment	HSD17B4	0.78
66	Nervous system disease	Enrichment	HSD17B4	0.78

¹ WikiPathways

² PubChem

³ R&D Systems

⁴ Reactome

⁵ GeneGo (Thomson Reuters)

⁶ QIAGEN

⁷ PharmGKB

⁸ Cell Signaling Technology

⁹ Sino Biological

¹⁰ Tocris

¹¹ MalaCards

Bile acid and bile salt metabolism

Pathway Network for Bile acid and bile salt metabolism

Pathway network for the Bile acid and bile salt metabolism SuperPath

Member Pathways for Bile acid and bile salt metabolism

Pathways in the Bile acid and bile salt metabolism SuperPath

Gene overlap in member pathways for Bile acid and bile salt metabolism SuperPath

Associated Genes for Bile acid and bile salt metabolism

Associated Disorders for Bile acid and bile salt metabolism

Disorders associated with Bile acid and bile salt metabolism SuperPath

STRING Interaction Network for Bile acid and bile salt metabolism

Sources for Bile acid and bile salt metabolism