HNF1B - HNF1 homeobox B Gene

Also Known as T2D; FJHN; HNF2; LFB3; RCAD; TCF2; HPC11; LF-B3; MODY5; TCF-2; VHNF1; ADTKD3; HNF-1B; HNF1beta; HNF-1-beta

Species: Homo sapiens

Gene Type: protein coding

Gene ID: 6928

About HNF1B

Cytogenetic location: 17q12 Genomic coordinates (GRCh38): 17:37,686,431-37,745,059 (from NCBI)

This gene has 5 transcripts (splice variants), 1 gene allele, 220 orthologues, 1 paralogue and is associated with 12 phenotypes. Biased expression in kidney (RPKM 16.9), gall bladder (RPKM 7.3) and 11 other tissues.

Summary

This gene encodes a member of the homeodomain-containing superfamily of transcription factors. The protein binds to DNA as either a homodimer, or a heterodimer with the related protein hepatocyte nuclear factor 1-alpha. The gene has been shown to function in nephron development, and regulates development of the embryonic pancreas. Mutations in this gene result in renal cysts and diabetes syndrome and noninsulin-dependent diabetes mellitus, and expression of this gene is altered in some types of Cancer. Multiple transcript variants encoding different isoforms have been found for this gene.[provided by RefSeq, Sep 2009]

HNF1B Products (4)

mRNA	Protein	Name
NM_000458.4	NP_000449.1	hepatocyte nuclear factor 1-beta isoform 1
NM_001165923.4	NP_001159395.1	hepatocyte nuclear factor 1-beta isoform 2
NM_001304286.2	NP_001291215.1	hepatocyte nuclear factor 1-beta isoform 3
NM_001411100.1	NP_001398029.1	hepatocyte nuclear factor 1-beta isoform 4

Gene Ontology

Molecular Function
Biological Process
Cellular Component

Molecular Function GO Annotation	Evidence	References	Source
enables DNA binding	IDA IDA: Inferred from direct assay	1677179	GOA
enables DNA-binding transcription factor activity	IDA IDA: Inferred from direct assay	15509593	GOA
enables protein binding	IPI IPI: Inferred from physical interaction	1677179	GOA
enables protein homodimerization activity	IPI IPI: Inferred from physical interaction	1677179	GOA
enables transcription coregulator binding	IPI IPI: Inferred from physical interaction	15509593	GOA

Biological Process GO Annotation	Evidence	References	Source
involved in endocrine pancreas development	IMP IMP: Inferred from mutant phenotype	16801329	GOA
involved in genitalia development	IMP IMP: Inferred from mutant phenotype	16801329	GOA
involved in kidney development	IDA IDA: Inferred from direct assay	21281489	GOA
involved in kidney development	IMP IMP: Inferred from mutant phenotype	10720943	GOA
involved in positive regulation of DNA-templated transcription	IDA IDA: Inferred from direct assay	15509593	GOA
involved in positive regulation of transcription initiation by RNA polymerase II	IDA IDA: Inferred from direct assay	15355349	GOA
involved in pronephric nephron tubule development	IGI IGI: Inferred from genetic interaction	15355349	GOA
involved in pronephros development	IMP IMP: Inferred from mutant phenotype	15355349	GOA
involved in regulation of pronephros size	IMP IMP: Inferred from mutant phenotype	15355349	GOA

Cellular Component GO Annotation	Evidence	References	Source
located in nucleus	IDA IDA: Inferred from direct assay	15355349	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

HNF1B Protein Structure

HNF-1_N

Homeobox

HNF-1B_C

0
100
200
300
400
500
557 a.a.

Protein Preferred Names	Protein Names
hepatocyte nuclear factor 1-beta	HNF1 beta A

HNF1B Protein-protein interaction Information

Type	Protein Name	Protein ID	Interactor	Interactor Species	Interactor ID	Detection Method	References
Intra	HNF1B	P35680	USP54	Homo sapiens	Q70EL1-9	Y2H Prey Pooling	32296183
Intra	HNF1B	P35680	USP54	Homo sapiens	Q70EL1-9	Validated Y2H	32296183
Intra	HNF1B	P35680	USP54	Homo sapiens	Q70EL1-9	Y2H Array	32296183
Intra	HNF1B	P35680	POU6F2	Homo sapiens	P78424	Validated Y2H	32296183
Intra	HNF1B	P35680	POU6F2	Homo sapiens	P78424	Y2H Array	32296183
Intra	HNF1B	P35680	POU6F2	Homo sapiens	P78424	Y2H Prey Pooling	32296183
Intra	HNF1B	P35680	UFSP1	Homo sapiens	Q6NVU6	Validated Y2H	32296183
Intra	HNF1B	P35680	UFSP1	Homo sapiens	Q6NVU6	Y2H Array	32296183
Intra	HNF1B	P35680	UFSP1	Homo sapiens	Q6NVU6	Y2H Prey Pooling	32296183
Intra	HNF1B	P35680	C10orf55	Homo sapiens	Q5SWW7	Validated Y2H	32296183
Intra	HNF1B	P35680	C10orf55	Homo sapiens	Q5SWW7	Y2H Array	32296183
Intra	HNF1B	P35680	C10orf55	Homo sapiens	Q5SWW7	Y2H Prey Pooling	32296183
Intra	HNF1B	P35680	OXER1	Homo sapiens	Q8TDS5	Y2H Array	32296183
Intra	HNF1B	P35680	OXER1	Homo sapiens	Q8TDS5	Y2H Prey Pooling	32296183
Intra	HNF1B	P35680	OXER1	Homo sapiens	Q8TDS5	Validated Y2H	32296183
Intra	HNF1B	P35680	MLLT10	Homo sapiens	P55197-2	Y2H Prey Pooling	32296183
Intra	HNF1B	P35680	MLLT10	Homo sapiens	P55197-2	Validated Y2H	32296183
Intra	HNF1B	P35680	MLLT10	Homo sapiens	P55197-2	Y2H Array	32296183
Intra	HNF1B	P35680	VAC14	Homo sapiens	Q08AM6	Y2H Array	32296183
Intra	HNF1B	P35680	VAC14	Homo sapiens	Q08AM6	Y2H Prey Pooling	32296183
Intra	HNF1B	P35680	VAC14	Homo sapiens	Q08AM6	Validated Y2H	32296183
Intra	HNF1B	P35680	KRTAP26-1	Homo sapiens	Q6PEX3	Y2H Array	32296183
Intra	HNF1B	P35680	KRTAP26-1	Homo sapiens	Q6PEX3	Y2H Prey Pooling	32296183
Intra	HNF1B	P35680	KRTAP26-1	Homo sapiens	Q6PEX3	Validated Y2H	32296183
Intra	HNF1B	P35680	HNF1A	Homo sapiens	P20823	Anti Tag CoIP	33961781
Intra	HNF1B	P35680	HNF1A	Homo sapiens	P20823	Affinity Chrom	35140242
Intra	HNF1B	P35680	PCBD1	Homo sapiens	P61457	Validated Y2H	32296183
Intra	HNF1B	P35680	PCBD1	Homo sapiens	P61457	Y2H Prey Pooling	32296183
Intra	HNF1B	P35680	PCBD1	Homo sapiens	P61457	Y2H Array	32296183
Intra	HNF1B	P35680	PCBD1	Homo sapiens	P61457	BioID	35140242
Intra	HNF1B	P35680	PCBD1	Homo sapiens	P61457	Affinity Chrom	35140242
Intra	HNF1B	P35680	CRX	Homo sapiens	O43186	Y2H Prey Pooling	32296183
Intra	HNF1B	P35680	CRX	Homo sapiens	O43186	Y2H Array	32296183
Intra	HNF1B	P35680	OTX2	Homo sapiens	P32243-2	Y2H Prey Pooling	32296183
Intra	HNF1B	P35680	OTX2	Homo sapiens	P32243-2	Y2H Array	32296183
Intra	HNF1B	P35680	BPIFA1	Homo sapiens	Q9NP55	Validated Y2H	32296183
Intra	HNF1B	P35680	BPIFA1	Homo sapiens	Q9NP55	Y2H Prey Pooling	32296183
Intra	HNF1B	P35680	BPIFA1	Homo sapiens	Q9NP55	Y2H Array	32296183

Cross: Cross-species interaction Intra: Intraspecies interaction

HNF1B Antibodies

Cat. No.	Product Name	Application	Reactivity
HY-P81979	HNF 1 beta Antibody (YA1724)	WB	Human
HY-P84278	HNF 1 beta Antibody (YA3975)	WB, IHC-P, ICC/IF, FC, ELISA	Human, Mouse, Rat
HY-P84278A	HNF 1 beta Antibody (YA3975)(PBS only)	WB, IHC-P, ICC/IF, FC, ELISA	Human, Mouse, Rat

Related Diseases

Diseases	Alias
Renal Cysts And Diabetes Syndrome	RCAD Mody5 Congenital Anomalies Of The Kidney And Urinary Tract With Diabetes Cakut With Diabetes Maturity-Onset Diabetes Of The Young Type 5 Tubulointerstitial Kidney Disease, Autosomal Dominant, 3 Adtkd3 Atypical Familial Juvenile Hyperuricemic Nephropathy Atypical Fjhn Familial Hypoplastic Glomerulocystic Kidney Maturity-Onset Diabetes Of The Young, Type 5 Hyperuricemic Nephropathy, Familial Juvenile, Atypical Fjhn, Atypical Glomerulocystic Kidney Disease, Hypoplastic Type Glomerulocystic Kidney, Familial Hypoplastic Hypoplastic Type Glomerulocystic Kidney Disease Glomerulocystic Kidney Disease Hypoplastic Type Renal-Diabetes Mody5 Syndrome
Type 2 Diabetes Mellitus	Insulin Resistance NIDDM Type 2 Diabetes Diabetes Mellitus, Non-Insulin-Dependent 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
Renal Cell Carcinoma, Nonpapillary	Renal Cell Carcinoma RCC Nonpapillary Renal Cell Carcinoma Clear Cell Renal Cell Carcinoma Hypernephroma Adenocarcinoma Of Kidney Renal Carcinoma, Chromophobe, Somatic Clear Cell Carcinoma Of Kidney Clear-Cell Metastatic Renal Cell Carcinoma Clear Cell Renal Carcinoma Renal Cell Carcinoma, Somatic Conventional Renal Cell Carcinoma Conventional Renal Cell Carcinoma Renal Clear Cell Carcinoma Ccrcc Hereditary Clear Cell Renal Cell Carcinoma Carcinoma, Renal Cell Renal Cell Carcinoma, Clear Cell, Somatic Renal Cell Carcinoma, Clear Cell Clear Cell Kidney Carcinoma Clear Cell Rcc Cystic-Multilocular Variant Clear Cell Renal Cell Adenocarcinoma Hereditary Clear Cell Renal Cell Adenocarcinoma Common Renal Cell Carcinoma Crcc Renal Cell Carcinoma Non-Papillary Carcinoma Renal Cell Renal Cell Cancer Carcinoma, Renal Cell, Nonpapillary
Hyperuricemic Nephropathy, Familial Juvenile, 3	HNFJ3 Hyperuricemic Nephropathy, Familial Juvenile Type 3
Hnf1b-Related Autosomal Dominant Tubulointerstitial Kidney Disease	Adtkd-Hnf1b Hnf1b-Mody Hnf1b-Related Nephropathy Mody5 Maturity-Onset Diabetes Of The Young Type 5 Rcad Syndrome Renal Cysts And Diabetes Syndrome Renal Dysfunction-Early-Onset Diabetes Syndrome
Unilateral Multicystic Dysplastic Kidney	Unilateral Mcdk Unilateral Multicystic Renal Dysplasia
Bilateral Multicystic Dysplastic Kidney	Bilateral Mcdk Bilateral Multicystic Renal Dysplasia Bilateral Multicystic Dysplastic Kidneys Multicystic Renal Dysplasia, Bilateral
Renal Dysplasia, Unilateral	Kidney Dysplasia, Unilateral
Renal Dysplasia, Bilateral	Kidney Dysplasia, Bilateral
Medullary Sponge Kidney	Cacchi-Ricci Disease Msk Precalicial Canalicular Ectasia Cacchi Ricci Disease Cacchi-Ricci Syndrome Cystic Dilatation Of Renal Collecting Tubes Precalyceal Canalicular Ectasia Sponge Kidney Congenital Cystic Kidney Disease Msk - [Medullary Sponge Kidney] Sponge Kidney Nos
Tubulointerstitial Kidney Disease, Autosomal Dominant, 2	Medullary Cystic Kidney Disease 1 Mckd1 Mckd Medullary Cystic Kidney Disease Type 1 Autosomal Dominant Tubulointerstitial Kidney Disease ADTKD2 Admckd1 Polycystic Kidneys, Medullary Type Adtkd Autosomal Dominant Medullary Cystic Kidney Disease Adtkd-Muc1 Muc1-Related Autosomal Dominant Tubulointerstitial Kidney Disease Muci-Related Adtkd Medullary Cystic Kidney Disease Medullary Cystic Kidney Disease Type I Medullary Cystic Kidney Disease, Autosomal Dominant Autosomal Dominant Tubulointerstitial Kidney Disease Due To Muc1 Mutations Adtkd Due To Muc1 Mutations Autosomal Dominant Tubulointerstitial Kidney Disease, Muc1-Associated Muc1-Related Autosomal Dominant Medullary Cystic Kidney Disease Mucin-1 Kidney Disease Autosomal Dominant Interstitial Kidney Disease Familial Juvenile Hyperuricemic Nephropathy Muc1-Related Medullary Cystic Kidney Disease Autosomal Dominant Medullary Cystic Kidney Disease 1 Medullary Polycystic Kidneys Medullary Cystic Kidney Disease 1, Autosomal Dominant Kidney Disease, Cystic, Medullary, Type 1 Nephronophthisis - Medullary Cystic Disease
Chromophobe Renal Cell Carcinoma	Chromophobe Adenocarcinoma Chromophobe Carcinoma Of Kidney Kidney Chromophobe Renal Cell Carcinoma, Chromophobe Cell Crcc Chrcc Chromophobe Renal Cell Adenocarcinoma Chromophobe Renal Carcinoma Chromophobe Carcinoma
Cakut	Renal Or Urinary Tract Malformation Congenital Anomalies Of Kidney And Urinary Tract Congenital Anomaly Of Kidney And Urinary Tract Congenital Anomalies Of The Kidney And Urinary Tract Kidney And Urinary Tract, Anomalies, Congenital Renal Hypodysplasia, Nonsyndromic, 1
Prostate Cancer, Hereditary, 11	HPC11 Familial Prostate Cancer 11
Chromosome 17q12 Deletion Syndrome	17q12 Microdeletion Syndrome Del(17)(Q12) Monosomy 17q12
Maturity-Onset Diabetes Of The Young	MODY Maturity Onset Diabetes Mellitus In Young Mason-Type Diabetes Mason Type Diabetes Maturity Onset Diabetes Of The Young Mody Syndrome Diabetes Of The Young, Maturity-Onset
Cystic Kidney Disease	Renal Cyst Simple Renal Cyst Kidney Cysts Kidney Diseases, Cystic Renal Cysts Kidney Cyst Cystic Kidney Congenital Cystic Kidney Disease Cystic Kidney Diseases Bosniak 1 Cyst
Oligomeganephronia	Oligomeganephronic Renal Hypoplasia Oligomeganephronic Hypoplasia Of Kidney
Tubulointerstitial Kidney Disease, Autosomal Dominant, 1	Familial Juvenile Hyperuricemic Nephropathy Mckd2 Familial Juvenile Hyperuricemic Nephropathy Type 1 Fjhn Medullary Cystic Kidney Disease 2 Uromodulin-Associated Kidney Disease Medullary Cystic Kidney Disease Type 2 ADTKD1 Hnfj1 Glomerulocystic Kidney Disease With Hyperuricemia And Isosthenuria Adtkd-Umod Familial Juvenile Hyperuricemic Nephropathy 1 Umod-Related Adtkd Umod-Related Autosomal Dominant Tubulointerstitial Kidney Disease Hyperuricemic Nephropathy, Familial Juvenile, 1 Gouty Nephropathy, Familial Juvenile Medullary Cystic Kidney Disease 2, Autosomal Dominant Admckd2 Autosomal Dominant Tubulointerstitial Kidney Disease Due To Umod Mutations Adtkd Due To Umod Mutations Autosomal Dominant Tubulointerstitial Kidney Disease, Umod-Related Autosomal Dominant Medullary Cystic Kidney Disease Type 2 Umod-Associated Kidney Disease Uromodulin Kidney Disease Familial Gout-Kidney Disease Familial Gouty Nephropathy Umak Umod-Related Kidney Disease Uromodulin Storage Disease Fjhn1 Gouty Nephropathy Familial Juvenile Nephropathy Familial With Gout Hyperuricemic Nephropathy, Familial Juvenile 1 Hyperuricemic Nephropathy, Familial Juvenile Kidney Disease, Glomerulocystic, With Hyperuricemia And Isosthenuria Nephropathy, Hyperuricemic, Juvenile, Familial, Type 1 Kidney Disease, Cystic, Medullary, Type 2 Medullary Cystic Kidney Disease Type Ii Familial Juvenile Gout
Uterine Anomalies	Uterine Diseases Uterine Disease Uterine Abnormality
Prune Belly Syndrome	Eagle-Barrett Syndrome Abdominal Muscle Deficiency Syndrome PBS Abdominal Muscles, Absence Of, With Urinary Tract Abnormality And Cryptorchidism Egbrs Eagle-Barret Syndrome Urethral Obstruction Sequence Obrinsky Syndrome Triad Syndrome Obrisnksy Syndrome Euos Early Urethral Obstruction Sequence Renal Dysplasia Or Hydronephrosis, Oligohydramnios And Subsequent Lung Hypoplasia Due To Urethral Obstruction Absence Of Abdominal Muscles With Urinary Tract Abnormality And Cryptorchidism Abdomen Muscle Deficiency Syndrome Abdomen Muscular Deficiency Syndrome Abdominal Muscular Deficiency Syndrome Abdominal Muscle Aplasia Syndrome
Bicornuate Uterus	Uterus Bicornis Uterus Biforis Bifid Uterus
Diabetes Mellitus	Diabetes
Neonatal Diabetes	Neonatal Diabetes Mellitus Diabetes Mellitus Syndrome In Newborn Infant Ndm
Ovary Transitional Cell Carcinoma	Malignant Ovarian Transitional Cell Neoplasm Ovarian Transitional Cell Cancer Ovarian Transitional Cell Neoplasm Transitional Cell Carcinoma Of Ovary Ovarian Transitional Cell Carcinoma Malignant Ovarian Transitional Cell Tumor Ovarian Transitional Cell Tumor
Renal Glucosuria	Renal Glycosuria Familial Renal Glucosuria GLYS Glys1 Glycosuria, Renal Glucosuria, Renal Renal Diabetes Familial Renal Glycosuria Sglt2 Deficiency Gly Diabetes Renal
Polycystic Kidney Disease 4 With Or Without Polycystic Liver Disease	Autosomal Recessive Polycystic Kidney Disease Arpkd Polycystic Kidney Disease, Autosomal Recessive Polycystic Kidney And Hepatic Disease 1 Pkhd1 PKD4 Polycystic Kidney Disease 4 With Or Without Hepatic Disease Polycystic Kidney Disease, Infantile, Type I Polycystic Kidney Disease, Infantile Type Polycystic Kidney, Autosomal Recessive Pkd3, Formerly Polycystic Kidney Disease 4, With Or Without Hepatic Disease Arpkd/Chf Ar-Pkd Polycystic Kidney Disease 4, With Or Without Polycystic Liver Disease Infantile Polycystic Kidney Disease Type I Pkd3 Kidney, Polycystic, Disease, Type 4, With/Without Hepatic Disease Polycystic Kidney Disease 3, Autosomal Dominant
Small Cell Carcinoma	Small Cell Carcinoma, Intermediate Cell Intermediate Cell Small Cell Carcinoma Small Cell Carcinoma - Intermediate Cell Small Cell Carcinoma Of Lung Carcinoma, Small Cell
Prostate Cancer	Prostate Carcinoma Prostate Cancer, Familial Prostate Neoplasm Prostate Cancer, Somatic Prostate Cancer, Susceptibility To Prostatic Cancer Prostatic Neoplasms Hereditary Prostate Cancer Prostatic Neoplasm Cancer Of Prostate Carcinoma Of Prostate Familial Prostate Cancer Familial Prostate Carcinoma Malignant Tumor Of Prostate Malignant Neoplasm Of Prostate Prostate Cancer, Familial, Susceptibility To Malignant Tumor Of The Prostate Ngp - New Growth Of Prostate Tumor Of The Prostate Prostate Cancer, Hereditary Cancer Of The Prostate Malignant Neoplasm Of The Prostate Prostatic Carcinoma PC Prca Cancer, Prostate Malignant Prostatic Tumour Malignant Tumour Of Prostate Primary Prostate Cancer Primary Malignant Neoplasm Of Prostate Prostate Gland Cancer
Kidney Disease	Renal Failure Kidney Failure Kidney Diseases Nephropathy Abnormality Of The Kidney Impaired Renal Function Disease Renal Anomaly Kidney Dysfunction Renal Disease Nephropathies Renal Failure Adverse Event Abnormal Renal Function
Polycystic Kidney Disease	Polycystic Kidney Diseases Pkd Polycystic Renal Disease Kidney Disease, Polycystic Polycystic Kidney, Autosomal Dominant
Autosomal Dominant Polycystic Kidney Disease	Polycystic Kidney Disease, Adult Type Adpkd Polycystic Kidney Diseases Polycystic Kidney, Autosomal Dominant Congenital Biliary Ectasias Polycystic Kidney And Hepatic Disease 1 Polycystic Kidney Disease, Autosomal Dominant Kidney, Polycystic, Disease, Autosomal Dominant Adult Polycystic Kidney Disease Polycystic Kidney, Adult Type Apckd - [Autosomal Polycystic Kidney Disease]
Maturity-Onset Diabetes Of The Young, Type 4	Maturity-Onset Diabetes Of The Young Type 4 MODY4 Mody, Type Iv Mody Type 4 Mody, Type 4 Maturity-Onset Diabetes Of The Young 4 Mody-4 Diabetes Of The Young, Maturity-Onset, Type 4 Maturity-Onset Diabetes Of The Young, Type Iv
Ovarian Clear Cell Carcinoma	Clear-Cell Ovarian Carcinoma
Maturity-Onset Diabetes Of The Young, Type 3	Maturity-Onset Diabetes Of The Young Type 3 MODY3 Mody, Type Iii Mody Type 3 Mody, Type 3 Maturity-Onset Diabetes Of The Young 3 Mody-3 Diabetes Of The Young, Maturity-Onset, Type 3
Maturity-Onset Diabetes Of The Young, Type 6	MODY6 Maturity-Onset Diabetes Of The Young Type 6 Maturity-Onset Diabetes Of The Young 6 Mody Type 6 Mody, Type 6 Mody-6 Diabetes Of The Young, Maturity-Onset, Type 6
Maturity-Onset Diabetes Of The Young, Type 1	Maturity-Onset Diabetes Of The Young Type 1 MODY1 Mild Juvenile Diabetes Mellitus Mody, Type I Diabetes Mellitus Type 2 Mody Type 1 Mody, Type 1 Maturity-Onset Diabetes Of The Young 1 Mody-1 Diabetes Of The Young, Maturity-Onset, Type 1
Endometriosis	Endometriosis, Site Unspecified Endometrial Ectopia
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
Malignant Ciliary Body Melanoma	Malignant Melanoma Of Ciliary Body Melanoma Of The Ciliary Body
Ciliary Body Cancer	Malignant Neoplasm Of Ciliary Body Malignant Tumor Of Ciliary Body Malignant Tumor Of The Ciliary Body Neoplasm Of Ciliary Body Tumor Of The Ciliary Body Primary Malignant Neoplasm Of Ciliary Body Malignant Neoplasm Of Eyeball Malignant Neoplasm Of Uveal Tract Primary Malignant Neoplasm Of Sclera Primary Malignant Neoplasm Of Uveal Tract
Endometrial Clear Cell Adenocarcinoma	Clear Cell Carcinoma Of Endometrium
Cervical Endometrioid Adenocarcinoma	Endometrioid Carcinoma Of The Cervix Uteri
Polyhydramnios
Maturity-Onset Diabetes Of The Young, Type 2	Maturity-Onset Diabetes Of The Young Type 2 MODY2 Mody Glucokinase-Related Mody Type 2 Mody, Type 2 Mody, Glucokinase-Related Mody, Type Ii Maturity-Onset Diabetes Of The Young 2 Mody-2 Diabetes Of The Young, Maturity-Onset, Type 2 Diabetes Mellitus Autosomal Dominant Type Ii
Polycystic Liver Disease 1 With Or Without Kidney Cysts	Polycystic Liver Disease 1 PCLD1 Cyst Liver Disease, Polycystic, Type 1 Polycystic Liver Disease
Ovarian Cancer	Ovarian Carcinoma Ovarian Neoplasm Malignant Tumour Of Ovary Cancer Of The Ovary Epithelial Ovarian Cancer Neoplasm Of Ovary Ovarian Neoplasms Ovarian Cancers Malignant Neoplasm Of Ovary Primary Malignant Neoplasm Of Ovary Ovarian Cancer, Somatic Malignant Ovarian Tumor Ovary Neoplasm Primary Ovarian Cancer Tumor Of The Ovary Malignant Neoplasm Of The Ovary Malignant Tumor Of The Ovary Ovarian Malignant Tumor OC Ovarian Carcinomas Cancer, Ovarian Cancer Of Ovary Ovary Cancer Ca Ovary
Pancreatic Agenesis	Partial Pancreatic Agenesis Congenital Pancreatic Agenesis Partial Agenesis Of The Pancreas Agenesis, Pancreatic Pancreatic Agenesis, Congenital
Oncocytoma	Oxyphilic Adenoma Follicular Adenoma, Oxyphilic Cell Adenoma, Oxyphilic Hurthle Cell Tumor Oncocytic Neoplasm
Ovarian Clear Cell Adenocarcinoma
Prediabetes Syndrome	Prediabetes Impaired Glucose Tolerance Prediabetic State IGT Igt - [Impaired Glucose Tolerance] Impaired Glucose Tolerance With Unspecified Complication Impaired Glucose Tolerance Without Complication Abnormal Glucose Tolerance
Renal Hypoplasia
Wolfram Syndrome 1	WFS1 Didmoad Wfs Diabetes Mellitus And Insipidus With Optic Atrophy And Deafness Diabetes Insipidus And Mellitus With Optic Atrophy And Deafness Diabetes Insipidus And Mellitus With Optic Atrophy And Deafness Syndrome Wolfram Syndrome
Endometrioid Ovary Carcinoma	Endometrioid Carcinoma Of Ovary Endometrioid Carcinoma Ovary Ovarian Endometrioid Carcinoma Malignant Ovarian Endometrioid Tumor Ovarian Endometrioid Tumor
Clear Cell Adenocarcinoma	Mesonephroma Water-Clear Cell Adenocarcinoma Adenocarcinoma, Clear Cell Malignant Mesonephroma Mesonephroid Clear Cell Carcinoma Mesonephroma, Malignant Water-Clear Cell Carcinoma Wolffian Duct Neoplasm Adenocarcinoma Clear Cell Mesonephric Tumor
Krukenberg Carcinoma	Krukenberg Tumor Krukenberg Neoplasm Krukenberg'S Tumor
Blepharophimosis, Ptosis, And Epicanthus Inversus	Blepharophimosis, Ptosis, And Epicanthus Inversus Syndrome BPES Blepharophimosis Syndrome Blepharophimosis, Epicanthus Inversus, And Ptosis, Type 1 Blepharophimosis, Ptosis, Epicanthus Inversus Syndrome Bpes With Duane Retraction Syndrome Blepharophimosis, Ptosis, And Epicanthus Inversus Syndrome Type 2 Blepharophimosis, Ptosis, And Epicanthus Inversus Syndrome Type 1 Blepharophimosis, Epicanthus Inversus, And Ptosis, Type 2 Blepharophimosis-Ptosis-Epicanthus Inversus Syndrome Blepharophimosis-Ptosis-Epicanthus Inversus Syndrome Type 2 Bpes Type 2 Blepharophimosis-Ptosis-Epicanthus Inversus Syndrome Without Premature Ovarian Failure Blepharophimosis-Ptosis-Epicanthus Inversus Syndrome Plus 3q23 Microdeletion Syndrome Bpes Plus Blepharophimosis-Ptosis-Epicanthus Inversus Syndrome Type 1 Bpes Type 1 Blepharophimosis-Ptosis-Epicanthus Inversus Syndrome With Premature Ovarian Failure Blepharophimosis, Ptosis, Epicanthus Inversus Autosomal Dominant Bpes Type I Autosomal Recessive Bpes Type I Bpes Type I Bpes Type Ii Bpes Without Ovarian Failure Bpes With Ovarian Failure Blepharophimosis Syndrome Type 1 Blepharophimosis Syndrome Type 2
Fallopian Tube Clear Cell Adenocarcinoma	Clear Cell Carcinoma Of The Fallopian Tube
Mullerian Duct Aplasia, Unilateral Renal Agenesis, And Cervicothoracic Somite Anomalies	Murcs Association Mayer-Rokitansky-Kuster-Hauser Syndrome Type 2 Mrkh Syndrome Type 2 Mayer-Rokitansky-Küster-Hauser Syndrome Type 2 MURCS Klippel-Feil Deformity, Conductive Deafness, And Absent Vagina Atypical Mrkh Syndrome Mullerian Duct Aplasia-Renal Dysplasia-Cervical Somite Anomalies Syndrome Mayer-Rokitansky-Kuster-Hauser Syndrome, Type Ii Mrkh, Type Ii Mullerian Duct Aplasia, Unilateral Renal Aplasia, And Cervicothoracic Somite Dysplasia Klippel-Feil Deformity - Conductive Deafness - Absent Vagina Müllerian Aplasia - Renal Aplasia - Cervicothoracic Somite Dysplasia
Mayer-Rokitansky-Kuster-Hauser Syndrome	Mrkh Syndrome Rokitansky Syndrome Mullerian Aplasia Mrkh Anomaly Congenital Absence Of Uterus And Vagina Congenital Absence Of The Uterus And Vagina Genital Renal Ear Syndrome Mayer-Rokitansky-Küster-Hauser Syndrome Mullerian Dysgenesis Müllerian Agenesis Rokitansky Kuster Hauser Syndrome MRKH Mullerian Aplasia/Dysgenesis Von Mayer-Rokitansky-Kuster Anomaly Mrk Anomaly Uterus Bipartitus Solidus Rudimentarius Cum Vagina Solida Cauv Mullerian Agenesis Aplasia Of The Mullerian Ducts Mullerian Duct Failure Müllerian Aplasia Rokitansky-Kuster-Hauser Syndrome RKH SYNDROME
Maturity-Onset Diabetes Of The Young, Type 10	Maturity-Onset Diabetes Of The Young Type 10 MODY10 Maturity-Onset Diabetes Of The Young 10 Mody-10 Mody Type 10 Diabetes Of The Young, Maturity-Onset, Type 10
Papillorenal Syndrome	Renal Coloboma Syndrome Coloboma Of Optic Nerve With Renal Disease Renal-Coloboma Syndrome Optic Nerve Coloboma With Renal Disease Optic Coloboma, Vesicoureteral Reflux, And Renal Anomalies Renal-Coloboma Syndrome With Macular Abnormalities Congenital Anomalies Of The Kidney And Urinary Tract With Or Without Ocular Abnormalities Cakut With Or Without Ocular Abnormalities PAPRS Optic Coloboma, Vesicoureteral Reflux And Renal Anomalies Papillo-Renal Syndrome, Optic Nerve Coloboma With Renal Disease Coloboma-Ureteral-Renal Syndrome Oncr Optic Nerve Coloboma Renal Syndrome Rcs Papillo-Renal Syndrome Optic Coloboma Vesicoureteral Reflux And Renal Anomalies
Caroli Disease	Caroli Disease Isolated Congenital Polycystic Dilatation Of Intrahepatic Bile Ducts Cystic Dilatation Of The Intrahepatic Biliary Tree Caroli Syndrome Carolis Disease
Papillary Adenofibroma
Chromosome 17q12 Duplication Syndrome	17q12 Microduplication Syndrome Trisomy 17q12 17q12 Duplication 17q12 Microduplication Dup(17)(Q12) Recurrent Duplication Of 17q12 17q12 Duplication Syndrome 17q12 Recurrent Duplication
Mucocele Of Appendix	Appendicele Mucocele Appendicular Mucocele Appendiceal Mucocele
Alagille Syndrome 1	Alagille Syndrome Arteriohepatic Dysplasia Alagille-Watson Syndrome Cholestasis With Peripheral Pulmonary Stenosis Hepatic Ductular Hypoplasia Alagille Syndrome Due To A Jag1 Point Mutation ALGS1 Algs Aws Syndromic Bile Duct Paucity Cardiovertebral Syndrome Hepatofacioneurocardiovertebral Syndrome Paucity Of Interlobular Bile Ducts Watson-Miller Syndrome Alagille Syndrome Due To 20p12 Microdeletion Ahd Hepatic Ductular Hypoplasia, Syndromatic Watson Alagille Syndrome Alagille'S Syndrome Alagille Syndrome Due To Del(20)(P12) Alagille Syndrome Due To Monosomy 20p12 Alagille-Watson Syndrome Due To Monosomy 20p12 Arteriohepatic Dysplasia Due To Monosomy 20p12 Syndromic Bile Duct Paucity Due To Monosomy 20p12 Alagille-Watson Syndrome Due To A Jag1 Point Mutation Arteriohepatic Dysplasia Due To A Jag1 Point Mutation Syndromic Bile Duct Paucity Due To A Jag1 Point Mutation Alagille Syndrome, Type 1
Permanent Neonatal Diabetes Mellitus	Pndm Permanent Diabetes Mellitus Of Infancy Pdmi Neonatal Diabetes Mellitus, Permanent
Serous Cystadenocarcinoma	Serous Adenocarcinoma Cystadenocarcinoma, Serous Serous Carcinoma Cystadenocarcinoma Serous
Endometriosis Of Ovary	Ovarian Endometriosis
Polycystic Liver Disease	Autosomal Dominant Polycystic Liver Disease Isolated Polycystic Liver Disease Pcld Congenital Cystic Liver Disease Congenital Hepatic Cyst Fibrocystic Liver Disease Isolated Autosomal Dominant Polycystic Liver Disease Adpcld Liver Disease, Polycystic Multiple Cysts Of Liver Pld - [Polycystic Liver Disease] Polycystic Liver Disorder Polycystic Liver Congenital Polycystic Disease Of Liver Congenital Polycystic Liver Disease
Basal Cell Carcinoma	Basal Cell Cancer Basal Cell Neoplasm Basal Cell Carcinoma Of Skin Malignant Basal Cell Tumor Basal Cell Tumor Epithelioma Basal Cell Malignant Basal Cell Neoplasm Rodent Ulcer Carcinoma Basal Cell Neoplasms, Basal Cell Basal Cell Carcinomas Experimental Organism Basal Cell Carcinoma Nodulo-Ulcerative Basal Cell Carcinoma Basalioma Basal Cell Epithelioma Of Skin Bcc - [Basal Cell Carcinoma] Of Skin Rodent Ulcer Of Skin Rodent Ulcer Of Unspecified Site Basal Cell Epithelioma Of Unspecified Site
Polycystic Kidney Disease 2 With Or Without Polycystic Liver Disease	Polycystic Kidney Disease 2 PKD2 Polycystic Kidney Disease, Adult, Type Ii Apkd2 Polycystic Kidney Disease, Type 2 Adpkd2 Adult Polycystic Kidney Disease Type 2 Autosomal Dominant Polycystic Kidney Disease 2 Pkd-2 Polycystic Kidney Disease Adult Type Ii Polycystic Kidney Type 2 Autosomal Dominant Disease Kidney Disease, Polycystic, Type 2
Renal Hypodysplasia/Aplasia 1	Renal Agenesis Renal Adysplasia Renal Aplasia RHDA1 Hereditary Renal Aplasia Hra Hereditary Urogenital Adysplasia Hypodysplasia/Aplasia, Renal, Type 1 Congenital Absence Of Kidneys Syndrome Congenital Absence Of Kidney Aplastic Kidney
Fallopian Tube Endometrioid Adenocarcinoma	Endometrioid Carcinoma Of The Fallopian Tube Fallopian Tube Endometrioid Cancer Fallopian Tube Endometrioid Neoplasm Fallopian Tube Endometrioid Tumor
Oligohydramnios	Oligohydramnios - Delivered Antepartum Oligohydramnios Delivered Oligohydramnios Oligohydramnios, Antepartum Condition Or Complication Deficient Liquor Oligohydramnios, Unspecified Trimester Reduced Liquor Volume
Chronic Kidney Disease	Chronic Renal Disease Chronic Kidney Failure Ckd Chronic Renal Failure Kidney Failure, Chronic Chronic Renal Failure Syndrome Crf Renal Failure - Chronic Renal Failure Chronic Chronic Kidney Diseases Chronic Kidney Disease Stage 5 Ckd - [Chronic Kidney Disease] Crf - [Chronic Renal Failure] Chronic Kidney Impairment Chronic Renal Impairment Chronic Kidney Shutdown Chronic Hypoxic Kidney Failure Chronic Kidney Collapse Chronic Renal Insufficiency Chronic Kidney Toxaemia Chronic Kidney Hypofunction Chronic Renal Suppression Chronic Renal Failure, Stage 5 Ckd - [Chronic Kidney Disease] Stage 5 End Stage Kidney Failure End Stage Renal Failure End Stage Kidney Disease End Stage Renal Disease End Stage Chronic Renal Failure Esrf - [End Stage Renal Failure] Esrd - [End Stage Renal Diseases] Egfr - [Estimated Glomerular Filtration Rate] < 15 Ml/Min/1.73m²
Branchiootorenal Syndrome	Branchio-Oto-Renal Syndrome Bor Syndrome Branchiootorenal Dysplasia Melnick-Fraser Syndrome Branchiootorenal Spectrum Disorders Branchio-Otorenal Dysplasia Branchio Oto Renal Syndrome Branchiootorenal/Branchiootic Syndrome Bo Syndrome Bor Bos Branchio-Otorenal Syndrome Branchiootic Syndrome Branchiootorenal Syndrome Branchiootic Syndrome 1
Vesicoureteral Reflux	Vesico-Ureteral Reflux
Nephronophthisis	Medullary Cystic Disease Medullary Cystic Kidney Nph Nphp Kidney Disease, Cystic, Medullary

Diseases	Alias
Waldenstroem'S Macroglobulinemia	Waldenstroem'S Macroglobulinemia Waldenstroem'S Macroglobulinemia Macroglobulinemia Of Waldenstrom Lymphoplasmacytic Lymphoma With Igm Gammopathy Lymphoplasmacytic Lymphoma Waldenstroem'S Macroglobulinemia Waldenstroem'S Macroglobulinemia Macroglobulinemia Of Waldenstrom Lymphoplasmacytic Lymphoma With Igm Gammopathy Lymphoplasmacytic Lymphoma

Related Agents

Pre-clinical Phase

Pre-clinical Phase

Bioactive Molecules (2)

Cat. No.	Product Name	Effect	Purity	Rare Diseases
HY-RS06256	HNF1B Human Pre-designed siRNA Set A	Pre-designed Set	/	Unkown
HY-174668	Human HNF1B mRNA		/	Unkown

Orthologs Information

Species	Symbol	Source	ID
Canis familiaris	HNF1B	VGNC	VGNC:41724
Mus musculus	HNF1B	MGD	MGI:98505
Bos taurus	HNF1B	VGNC	VGNC:29890
Felis catus	HNF1B	VGNC	VGNC:62826
Macaca mulatta	HNF1B	VGNC	VGNC:73384
Rattus norvegicus	HNF1B	RGD	RGD:3830
Others	HNF1B	NCBI