NR1H4 - nuclear receptor subfamily 1 group H member 4 Gene

Homo sapiens

Also known as BAR; FXR; HRR1; HRR-1; PFIC5; RIP14

Gene ID: 9971 | Gene type: protein coding

About NR1H4

Cytogenetic location: 12q23.1 Genomic coordinates (GRCh38): 12:100,473,866-100,564,414 (from NCBI)

This gene has 11 transcripts (splice variants), 388 orthologues, 18 paralogues and is associated with 3 phenotypes. Biased expression in liver (RPKM 14.7), small intestine (RPKM 8.3) and 6 other tissues.

Summary

This gene encodes a ligand-activated transcription factor that shares structural features in common with nuclear hormone receptor family members. This protein functions as a receptor for bile acids, and when bound to bile acids, binds to DNA and regulates the expression of genes involved in bile acid synthesis and transport. Alternatively spliced transcript variants encoding different isoforms have been described. [provided by RefSeq, Feb 2016]

NR1H4 Products(12)

mRNA	Protein	Name
NM_005123.4	NP_005114.1	bile acid receptor isoform 2
NR_135146.2
NM_001206979.2	NP_001193908.1	bile acid receptor isoform 1
XM_006719719.3	XP_006719782.1	bile acid receptor isoform X4
XM_011539040.3	XP_011537342.1	bile acid receptor isoform X1
NM_001206977.2	NP_001193906.1	bile acid receptor isoform 1
NM_001206978.2	NP_001193907.1	bile acid receptor isoform 5
NM_001206993.2	NP_001193922.1	bile acid receptor isoform 3
NM_001206992.2	NP_001193921.1	bile acid receptor isoform 4
XM_047429943.1	XP_047285899.1	bile acid receptor isoform X2
XM_047429944.1	XP_047285900.1	bile acid receptor isoform X5
XM_011539041.3	XP_011537343.1	bile acid receptor isoform X3

Gene Ontology

Molecular Function
Biological Process
Cellular Component

Molecular Function GO Annotation	Evidence	Reference	Source
enables DNA-binding transcription activator activity, RNA polymerase II-specific	IDA IDA: Inferred from direct assay	21757002	GOA
enables DNA-binding transcription factor activity, RNA polymerase II-specific	IDA IDA: Inferred from direct assay	21757002	GOA
enables RNA polymerase II cis-regulatory region sequence-specific DNA binding	IDA IDA: Inferred from direct assay	21757002	GOA
enables RNA polymerase II transcription regulatory region sequence-specific DNA binding	IDA IDA: Inferred from direct assay	23767959	GOA
enables bile acid binding	IDA IDA: Inferred from direct assay	21757002	GOA
enables bile acid nuclear receptor activity	IDA IDA: Inferred from direct assay	10334992	GOA
enables chenodeoxycholic acid binding	IDA IDA: Inferred from direct assay	10334992	GOA
enables nuclear receptor activity	IDA IDA: Inferred from direct assay	27471003	GOA
enables protein binding	IPI IPI: Inferred from physical interaction	12917342	GOA
enables sequence-specific DNA binding	IDA IDA: Inferred from direct assay	12815072	GOA
enables transcription cis-regulatory region binding	IMP IMP: Inferred from mutant phenotype	26888176	GOA
enables transcription coregulator binding	IPI IPI: Inferred from physical interaction	14701856	GOA

Biological Process GO Annotation	Evidence	Reference	Source
involved in cellular response to bile acid	IDA IDA: Inferred from direct assay	10334992	GOA
involved in cellular response to bile acid	IMP IMP: Inferred from mutant phenotype	23767959	GOA
involved in cellular response to fatty acid	IDA IDA: Inferred from direct assay	20447400	GOA
involved in intracellular triglyceride homeostasis	IDA IDA: Inferred from direct assay	20447400	GOA
involved in negative regulation of apoptotic process	IDA IDA: Inferred from direct assay	20447400	GOA
involved in negative regulation of canonical NF-kappaB signal transduction	IDA IDA: Inferred from direct assay	21242261	GOA
involved in negative regulation of tumor necrosis factor production	IDA IDA: Inferred from direct assay	21242261	GOA
involved in negative regulation of type II interferon production	IDA IDA: Inferred from direct assay	21242261	GOA
acts upstream of or within negative regulation of very-low-density lipoprotein particle remodeling	IDA IDA: Inferred from direct assay	27471003	GOA
involved in nuclear receptor-mediated bile acid signaling pathway	IDA IDA: Inferred from direct assay	10334992	GOA
acts upstream of or within positive regulation of DNA-templated transcription	IDA IDA: Inferred from direct assay	27471003	GOA
involved in positive regulation of interleukin-17 production	IDA IDA: Inferred from direct assay	21242261	GOA
involved in positive regulation of phosphatidic acid biosynthetic process	IDA IDA: Inferred from direct assay	23767959	GOA
involved in positive regulation of transcription by RNA polymerase II	IDA IDA: Inferred from direct assay	23767959	GOA
involved in positive regulation of transcription by RNA polymerase II	IMP IMP: Inferred from mutant phenotype	21757002	GOA
involved in regulation of DNA-templated transcription	IMP IMP: Inferred from mutant phenotype	26888176	GOA
involved in regulation of insulin secretion involved in cellular response to glucose stimulus	IDA IDA: Inferred from direct assay	20447400	GOA
involved in regulation of low-density lipoprotein particle clearance	IDA IDA: Inferred from direct assay	12660231	GOA
involved in regulation of transcription by RNA polymerase II	IDA IDA: Inferred from direct assay	10334992	GOA

Cellular Component GO Annotation	Evidence	Reference	Source
located in euchromatin	IDA IDA: Inferred from direct assay	21757002	GOA
part of receptor complex	IDA IDA: Inferred from direct assay	10334992	GOA

EXP: Inferred from Experiment IDA: Inferred from direct assay IPI: Inferred from physical interaction IMP: Inferred from mutant phenotype IGI: Inferred from genetic interaction IEP: Inferred from expression pattern

NR1H4 Protein Structure

zf-C4

Hormone_recep

0
100
200
300
400
486 a.a.

Protein Preferred Names

Protein Names

bile acid receptor

RXR-interacting protein 14	farnesoid X nuclear receptor
farnesoid X-activated receptor	farnesol receptor HRR-1
retinoid X receptor-interacting protein 14

NR1H4 Protein-protein interaction Information

Type Cross Intra	Protein Name	Protein ID	Interactor	Interactor Species	Interactor ID	Detection Method	Reference
Intra	NR1H4	Q96RI1	PHF2	Homo sapiens	O75151	Pull Down	21532585
Intra	NR1H4	Q96RI1	SETD7	Homo sapiens	Q8WTS6	Y2H	22345554
Intra	NR1H4	Q96RI1	SETD7	Homo sapiens	Q8WTS6	Anti Bait CoIP	22345554
Intra	NR1H4	Q96RI1	SETD7	Homo sapiens	Q8WTS6	Pull Down	22345554
Intra	NR1H4	Q96RI1	SETD7	Homo sapiens	Q8WTS6	Methyltransferase Ass	22345554
Intra	NR1H4	Q96RI1	NCOA1	Homo sapiens	Q15788	HTRF	14684751
Intra	NR1H4	Q96RI1	NCOA1	Homo sapiens	Q15788	Pull Down	14684751

Cross: Cross-species interaction Intra: Intraspecies interaction

Related Diseases

Diseases

Alias

Biliary Tract Disease

Biliary Tract Diseases

Biliary Tract Abnormality

Cardiovascular System Disease

Abnormality Of The Cardiovascular System	Disease Of Subdivision Of Hemolymphoid System
Disorder Of Cardiovascular System	Cardiovascular Diseases
Cardiovascular Disease

Cholestasis

Obstruction Of Bile Duct	Bile Duct Obstruction
Bile Occlusion	Extrahepatic Biliary Obstruction
Extrahepatic Bile Duct Obstruction	Bile Stasis
Biliary Stasis	Obstructive Hyperbilirubinemia
Obstructed Jaundice	Bile Duct Obstructed
Bile Ductal Obstruction	Biliary Duct Obstruction
Obstructed Bile Ductal	Obstructed Biliary Duct
Obstructed Biliary Ductal	Jaundice Regurgitation
Obstructive Jaundice	Cholestatic Jaundice
Cholestatic Jaundice Syndrome

Body Mass Index Quantitative Trait Locus 11

OBESITY	Obesity, Susceptibility To
Leanness, Inherited	Obesity, Susceptibility To, Bmiq11
Obesity, Mild, Early-Onset	Obesity, Association With
Obesity, Early-Onset, Susceptibility To	Obesity, Severe
Obesity, Severe, And Type Ii Diabetes	Obesity, Late-Onset
BMIQ11	Obesity Bmiq11
Obesity, Early-Onset	Obesity , Susceptibility To
Simple Obesity Nos	Excess Fat
Obesity, Not Elsewhere Classified, Body Mass Index Not Elsewhere Classified	Adiposis

Lysosomal Storage Disease

Lysosomal Storage Diseases	Disorder Of Lysosomal Enzyme
Inborn Lysosomal Enzyme Disorder	Lysosomal Storage Metabolism Disorder
Lysosomal Storage Disorder

Osteogenesis Imperfecta, Type Xiii

Osteogenesis Imperfecta Type 13	OI13
Osteogenesis Imperfecta Type Xiii	Oi, Type Xiii
Osteogenesis Imperfecta 13	Oi Type Xiii
Oi-Xiii

Cholangitis

Acute Cholangiolitis	Ascending Cholangitis
Cholangiolitis	Cholangitis Nos
Chronic Cholangiolitis	Hepatic Duct Inflammation
Acute Cholangitis	Bile Duct Inflammation

Leber Congenital Amaurosis 7

LCA7	Leber Congenital Amaurosis, Type 7

Fatty Liver Disease

Alcoholic Fatty Liver	Fatty Liver
Fatty Liver, Alcoholic	Fatty Change Of Liver
Hepatic Lipidosis	Steatosis Of Liver
Fatty Liver Alcoholic	Steatohepatitis
Etoh Fatty Liver	Etoh Fatty Liver Metamorphosis
Fatty Etoh Liver Necrosis

Cholestasis, Progressive Familial Intrahepatic, 5

PFIC5	Progressive Familial Intrahepatic Cholestasis 5
Nr1h4 Deficiency	Progressive Familial Intrahepatic Cholestasis Type 5
Cholestasis, Intrahepatic, Familial, Progressive, Type 5

Bilirubin Metabolic Disorder

Hyperbilirubinemia	Hereditary Hyperbilirubinemia
Hyperbilirubinemia, Hereditary	Hyperbilirubinaemia

Primary Biliary Cholangitis

Primary Biliary Cirrhosis	Biliary Liver Cirrhosis
Chronic Nonsuppurative Destructive Cholangitis	Familial Primary Biliary Cirrhosis
Pbc	Hanot Syndrome
Cholestatic Cirrhosis	Biliary Cirrhosis Primary
Liver Cirrhosis, Biliary	Hanot'S Cirrhosis
Biliary Cirrhosis	Pericholangiolic Biliary Cirrhosis
Tannhauser-Magendantz Syndrome	Hanot-Rossle Syndrome
Hypertrophic Cirrhosis	Todd Cirrhosis
Hanot Cirrhosis	Charcot Cirrhosis
Mahon-Tannhauser Syndrome	Toxic Cirrhosis
Hypertrophic Biliary Cirrhosis	Monolobular Cirrhosis
Unilobar Cirrhosis	Xanthomatous Biliary Cirrhosis

Bile Duct Disease

Bile Duct Diseases	Bile Duct Disorder
Disorder Of Bile Duct

Functional Diarrhea

Functional Diarrhoea

Cholestasis, Progressive Familial Intrahepatic, 2

PFIC2	Cholestasis, Progressive Familial Intrahepatic 2
Progressive Familial Intrahepatic Cholestasis Type 2	Progressive Familial Intrahepatic Cholestasis 2
Bsep Deficiency	Recurrent Familial Intrahepatic Cholestasis 2
Benign Recurrent Intrahepatic Cholestasis 2	Severe Abcb11 Deficiency
Bric2	Cholestasis, Benign Recurrent Intrahepatic 2
Mild Abcb11 Deficiency	Cholestasis, Intrahepatic, Familial, Progressive, Type 2

Liver Disease

Liver Failure	Liver Diseases
Abnormality Of The Liver	Liver Dysfunction
Disorder Of Liver	Hepatic Disorder
Hepatic Disease	Disease Of Bilirubin Metabolism
Disorder Of Bilirubin Metabolism	Liver Decompensation
Liver Function Failure	Hepatic Failure Nos
Liver Failure Nos	End Stage Liver Disease
Decompensated Liver Failure	Decompensation Of Liver Function
Hepatic Decompensation	Hepatic Insufficiency
Liver Cell Necrosis With Hepatic Failure	Liver Insufficiency
Decompensated Liver Disease	End Stage Liver Failure
Liver Necrosis With Hepatic Failure

Diabetes Mellitus

Diabetes

Hypercholesterolemia, Familial, 1

Hypercholesterolemia	FHCL1
Fhc	Fh
Hyperlipoproteinemia, Type Ii	Hyperlipoproteinemia, Type Iia
Hyper-Low-Density-Lipoproteinemia	Hypercholesterolemic Xanthomatosis, Familial
Ldl Receptor Disorder	Hypercholesterolemia, Susceptibility To
Hypercholesterolemia, Familial, Modifier Of	Hypercholesterolemia, Familial, Due To Ldlr Defect, Modifier Of
Ldl Cholesterol Level Qtl2	Hyperlipoproteinemia Type Ii
Hypercholesterolemia, Familial, Type 1	High Cholesterol
Increased Cholesterol	Low-Density-Lipoid-Type Hyperlipoproteinemia
Pure Hypercholesterolaemia	Ldl - [Low Density Lipoprotein} Hyperlipoproteinemia
Group A Hyperlipidaemia	Pure Hypercholesterinaemia
Cholesterolaemia	Essential Cholesterolaemia
Essential Hypercholesterolaemia	Group A Hyperlipemia
Increased Low Density Lipoprotein	Low-Density-Lipoprotein-Type
Low-Density-Lipoprotein-Type Hyperlipoproteinemia

Type 2 Diabetes Mellitus

Insulin Resistance	NIDDM
Diabetes Mellitus, Non-Insulin-Dependent	Type 2 Diabetes
T2D	Noninsulin-Dependent Diabetes Mellitus
Diabetes Mellitus, Type Ii	Maturity-Onset Diabetes
Insulin Resistance, Severe, Digenic	Diabetes Mellitus, Type 2
Diabetes Mellitus, Noninsulin-Dependent	Diabetes Mellitus, Noninsulin-Dependent, Association With
Diabetes Mellitus, Noninsulin-Dependent, Late Onset	Hypertension, Insulin Resistance-Related, Susceptibility To
Insulin Resistance, Susceptibility To	Non-Insulin-Dependent Diabetes Mellitus
Type Ii Diabetes Mellitus	Adult-Onset Diabetes Mellitus
Maturity-Onset Diabetes Mellitus	Diabetes Mellitus Type 2
Type Ii Diabetes	Type 2 Diabetes Mellitus, Susceptibility To
Diabetes, Type 2	Diabetes Mellitus, Noninsulin-Dependent, Susceptibility To
Diabetes Mellitus, Non-Insulin-Dependent, Susceptibility To	Diabetes Mellitus, Type 2, Susceptibility To
Diabetes Mellitus, Noninsulin-Dependent, 2	Diabetes Mellitus, Type Ii, Susceptibility To
Hypertension, Insulin Resistance-Related	Adult-Onset Diabetes
Aodm	Diabetes Mellitus, Adult-Onset
Diabetes Mellitus Type Ii	Diabetes Mellitus Type 2, Susceptibility To
Diabetes, Type Ii, Susceptibility To	Diabetes Type 2
Diabetes Mellitus	Adult Onset Diabetes
Maturity Onset Diabetes	Nonketotic Diabetes
Non-Insulin Dependent Diabetes Mellitus	T2dm - [Type 2 Diabetes Mellitus]
Niddm - [Non Insulin Dependent Diabetes Mellitus]	Dm2
Dm Type Ii	Diabetic Type 2
Insulin Requiring Type 2 Diabetes	Noninsulin Dependent Diabetes
Non-Insulin-Dependent Diabetes Mellitus Without Complications	Diabetes Due To Insulin Secretory Defect
Diabetes Mellitus Due To Insulin Secretory Defect	Non-Insulin-Dependent Diabetes Of The Young
Senile Diabetes	Nonketotic Hyperglycaemia
Stable Diabetes

Cholelithiasis

Abdominal Obesity-Metabolic Syndrome 1

Metabolic Syndrome X	Metabolic Syndrome
AOMS1	Dysmetabolic Syndrome X
Metabolic Disease	Abdominal Obesity Metabolic Syndrome

Non-Alcoholic Fatty Liver Disease

Fatty Liver	Non-Alcoholic Fatty Liver
Nafld	Nonalcoholic Fatty Liver Disease
Nonalcoholic Steatohepatitis	Steatosis
Nafl	Nash
Non-Alcoholic Steatohepatitis	Susceptibility To Nonalcoholic Fatty Liver Disease
Steatohepatitis	Fatty Degeneration
Non-Alcoholic Fatty Liver Disease Without Mention Of Non-Alcoholic Steatohepatitis	Nafld Without Nash
Nafld Without Mention Of Nash

Cerebrotendinous Xanthomatosis

CTX	Cerebral Cholesterinosis
Cholestanol Storage Disease	Xanthomatosis, Cerebrotendinous
Sterol 27-Hydroxylase Deficiency	Xanthomatosis Cerebrotendinous
Cerebrotendinous Cholesterinosis	Cholestanolosis
Van Bogaert-Scherer-Epstein Disease

Trimethylaminuria

TMAU	Fish-Odor Syndrome
Fish Malodor Syndrome	Fish Odor Syndrome
Stale Fish Syndrome	Tmauria
Severe Primary Trimethylaminuria	Mesh
D008661	Fish Odour Syndrome

Sclerosing Cholangitis

Fibrosing Cholangitis	Cholangitis, Sclerosing
Primary Sclerosing Cholangitis

Fragile X Syndrome

FXS	Martin-Bell Syndrome
Fraxa Syndrome	Marker X Syndrome
X-Linked Mental Retardation And Macroorchidism	Fragile X Mental Retardation Syndrome
Fra Syndrome	Mental Retardation, X-Linked, Associated With Marxq28
Frax Syndrome	Symptomatic Form Of Fragile X Syndrome In Female Carriers
Fragile-X Syndrome	Fraxe Syndrome

Cholangitis, Primary Sclerosing

Primary Sclerosing Cholangitis	PSC
Sclerosing Cholangitis	Cholangitis, Sclerosing
Cholangitis Primary Sclerosing	Psc - [Primary Sclerosing Cholangitis]

Non-Alcoholic Steatohepatitis

Nonalcoholic Steatohepatitis	Nash
Nash - [Non-Alcoholic Steatohepatitis]	Non-Alcoholic Steatohepatosis

Pericholangitis

Barrett Esophagus

Barrett Esophagus/Esophageal Adenocarcinoma	Barrett'S Esophagus
Barrett Metaplasia	Barrett'S Ulcer Of Esophagus
Ulcerative Esophagitis	Barrett'S Esophagus With Esophagitis
Barrett'S Oesophagus	Barretts Syndrome
Barrett Syndrome	BE
Peptic Ulcer Of Esophagus	Adenocarcinoma Of Esophagus

Biliary Atresia

Congenital Biliary Atresia	Isolated Biliary Atresia
Isolated Atresia Of Bile Ducts	Non-Syndromic Biliary Atresia
Atresia Of Bile Duct	Biliary Atresia, Congenital
Atresia Of Bile Ducts	Bile Duct Atresia
Congenital Bile Duct Atresia	Ba - [Biliary Atresia]
Impervious Bile Duct	Atresia Of Common Duct
Biliary Duct Atresia	Bile Ductal Atresia
Cystic Duct Atresia

Intrahepatic Cholestasis Of Pregnancy

Recurrent Intrahepatic Cholestasis Of Pregnancy	Gravidic Intrahepatic Cholestasis
Pregnancy-Related Cholestasis	Icp
Pregnancy Related Cholestasis	Cholestasis, Intrahepatic Of Pregnancy
Familial Intrahepatic Cholestasis Of Pregnancy	Familial Recurrent Intrahepatic Cholestasis Of Pregnancy
Ricp	Obstetric Cholestasis

Autoimmune Disease Of Gastrointestinal Tract

Hepatic Vascular Disease

Vascular Disorder Of Liver

Cholestasis, Benign Recurrent Intrahepatic, 1

Benign Recurrent Intrahepatic Cholestasis	BRIC1
Summerskill Syndrome	Bric
Summerskill-Walshe-Tygstrup Syndrome	Cholestasis, Benign Recurrent Intrahepatic
Benign Recurrent Intrahepatic Cholestasis 1	Benign Recurrent Intrahepatic Cholestasis Type 1
Bric Type 1	Low Gamma-Gt Familial Intrahepatic Cholestasis
Recurrent Familial Intrahepatic Cholestasis	Cholestasis, Benign Recurrent Intrahepatic 1
Mild Atp8b1 Deficiency	Recurrent Familial Intrahepatic Cholestasis 1
Abcb11-Related Intrahepatic Cholestasis	Atp8b1-Related Intrahepatic Cholestasis
Cholestasis, Intrahepatic, Recurrent, Benign, Type 1	Progressive Intrahepatic Cholestasis
Cholestasis, Progressive Familial Intrahepatic 3	Bric - [Benign Recurrent Intrahepatic Cholestasis]

Bile Reflux

Arthrogryposis, Distal, Type 1c

DA1C	Distal Arthrogryposis Type 1c
Arthrogryposis, Distal, 1c

Cholestasis, Benign Recurrent Intrahepatic, 2

BRIC2	Benign Recurrent Intrahepatic Cholestasis 2
Benign Recurrent Intrahepatic Cholestasis Type 2	Bric Type 2
Cholestasis, Intrahepatic, Recurrent, Benign, Type 2	Cholestasis, Benign Recurrent Intrahepatic 2

Lipid Metabolism Disorder

Dyslipidemia	Disorder Of Fatty Acid Metabolism
Lipid Metabolism Disorders	Fatty Acid Metabolism Disorder
Disorder Of Lipid Metabolism	Abnormality Of Lipid Metabolism
Lipid Metabolism, Inborn Errors	Dyslipidemias
Disorders Of Lipid Metabolism	Congenital Disorders Of Lipid Metabolism
Inherited Disorders Of Lipid Metabolism

Cholestasis, Progressive Familial Intrahepatic, 4

PFIC4	Progressive Familial Intrahepatic Cholestasis 4
Cholestasis, Progressive Familial Intrahepatic 4	Tjp2 Deficit
Progressive Familial Intrahepatic Cholestasis-4	3-Beta-Hydroxy-Delta-5-C27-Steroid Oxidoreductase Deficiency
Progressive Familial Intrahepatic Cholestasis Type 4	Cholestasis, Intrahepatic, Familial, Progressive, Type 4
Bile Acid Synthesis Defect, Congenital, 1

Cholestasis, Progressive Familial Intrahepatic, 1

PFIC1	Byler Disease
Cholestasis, Progressive Familial Intrahepatic 1	Progressive Familial Intrahepatic Cholestasis 1
Progressive Familial Intrahepatic Cholestasis Type 1	Fic1 Deficiency
Byler'S Disease	Cholestasis, Fatal Intrahepatic
Progressive Familial Intrahepatic Cholestasis	Severe Atp8b1 Deficiency
Fatal Intrahepatic Cholestasis	Cholestasis, Intrahepatic, Familial, Progressive, Type 1
Progressive Intrahepatic Cholestasis	Cholestasis, Progressive Familial Intrahepatic 3

Xanthomatosis

Xanthomatosis, Susceptibility To

Xanthelasmatosis

Choline Deficiency Disease

Choline Deficiency

Lipid Storage Disease

Lipoidosis	Inborn Lipid Storage Disorder
Lipoid Storage Diseas	Lipid Storage Diseases
Lipidoses

Portal Hypertension

Hypertension, Portal	Hypertension Portal
Pht - [Portal Hypertension]	Portal Htn

Alcoholic Hepatitis

Acute Alcoholic Hepatitis	Acute Alcoholic Liver Disease
Hepatitis, Alcoholic	Hepatitis Alcoholic
Ah - [Alcoholic Hepatitis]	Ethanol Hepatitis

Progressive Familial Intrahepatic Cholestasis

Abcb4-Related Intrahepatic Cholestasis	Cholestasis, Progressive Familial Intrahepatic
Pfic	Byler Disease
Abcb11-Related Intrahepatic Cholestasis	Atp8b1-Related Intrahepatic Cholestasis
Bsep Deficiency	Byler Disease
Byler Syndrome	Fic1 Deficiency
Low Γ-Gt Familial Intrahepatic Cholestasis	Mdr3 Deficiency
Pfic	Cholestasis, Intrahepatic, Familial, Progressive
Pfic - [Progressive Familial Intrahepatic Cholestasis]

Hepatocellular Clear Cell Carcinoma

Clear Cell Carcinoma Of The Liver Cells	Hepatocellular Carcinoma, Clear Cell Type
Clear Cell Hepatocellular Carcinoma

Extrahepatic Cholestasis

Cholestasis, Extrahepatic	Extrahepatic Biliary Stasis
Extrahepatic Obstructive Biliary Disease	Cholestasis Extrahepatic

Gallbladder Disease

Gallbladder Diseases	Gall Bladder
Gall Bladder Diseases	Abnormal Gallbladder Function

Cholestasis, Progressive Familial Intrahepatic, 3

PFIC3	Cholestasis, Progressive Familial Intrahepatic 3
Mdr3 Deficiency	Progressive Familial Intrahepatic Cholestasis Type 3
Progressive Familial Intrahepatic Cholestasis 3	Progressive Familial Intrahepatic Cholestasis With Elevated Serum Gamma-Glutamyltransferase
Cholestasis, Progressive Familial Intrahepatic, With Elevated Serum Gamma-Glutamyltransferase	Progressive Familial Intrahepatic Cholestasis With Elevated Serum Gama-Glutamyltransferase
Cholestasis, Intrahepatic, Familial, Progressive, Type 3

Related Agents

Pre-clinical Phase

Bioactive Molecules (4)

Cat. No.	Product Name	Effect	Purity	Rare Diseases
HY-125996	NR1H4 activator 1	Agonist	/	Unkown
HY-RS09531	NR1H4 Human Pre-designed siRNA Set A	Pre-designed Sets	/	Unkown
HY-RS09532	Nr1h4 Mouse Pre-designed siRNA Set A	Pre-designed Sets	/	Unkown
HY-RS09533	Nr1h4 Rat Pre-designed siRNA Set A	Pre-designed Sets	/	Unkown

Orthologs Information

Species	Symbol	Source	ID
Bos taurus	NR1H4	VGNC	VGNC:32232
Canis familiaris	NR1H4	VGNC	VGNC:43944
Felis catus	NR1H4	VGNC	VGNC:68546
Rattus norvegicus	NR1H4	RGD	RGD:628831
Mus musculus	NR1H4	MGD	MGI:1352464
Macaca mulatta	NR1H4	VGNC	VGNC:75506