HNF1B

Summary

HNF1B (HNF1 homeobox B, HGNC:11630) is a protein-coding gene on chromosome 17q12, encoding Hepatocyte nuclear factor 1-beta (P35680). Transcription factor that binds to the inverted palindrome 5’-GTTAATNATTAAC-3’. It is a selective cancer dependency (DepMap: 12.0% of cell lines) and haploinsufficient (ClinGen: sufficient evidence).

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.

Source: NCBI Gene 6928 — RefSeq curated summary.

At a glance

• Gene–disease (curated): renal cysts and diabetes syndrome (Definitive, ClinGen) — +8 more curated relationships
• GWAS associations: 60
• Clinical variants (ClinVar): 890 total — 155 pathogenic, 82 likely-pathogenic
• Phenotypes (HPO): 90
• Cancer dependency (DepMap): dependent in 12.0% of screened cell lines
• Dosage sensitivity (ClinGen): haploinsufficiency sufficient evidence, triplosensitivity no evidence
• Transcription factor: yes — 71 downstream targets (CollecTRI)
• MANE Select transcript: NM_000458

Identifiers

Gene identifiers

Field	Value
HGNC ID	HGNC:11630
Approved symbol	HNF1B
Name	HNF1 homeobox B
Location	17q12
Locus type	gene with protein product
Status	Approved
Aliases	LFB3, VHNF1, HNF1beta, MODY5, HNF1β
Ensembl gene	ENSG00000275410
Ensembl biotype	protein_coding
OMIM	189907
Entrez	6928

Gene structure

Transcript identifiers

Ensembl transcripts: 7 — 6 protein_coding, 1 retained_intron

ENST00000613727, ENST00000614313, ENST00000617811, ENST00000618894, ENST00000621123, ENST00000904916, ENST00000904917

RefSeq mRNA: 4 — MANE Select: NM_000458 NM_000458, NM_001165923, NM_001304286, NM_001411100

CCDS: CCDS11324, CCDS58538, CCDS77007, CCDS92293

Canonical transcript exons

ENST00000610754 — 0 exons

Expression profiles

Bgee: expression breadth broad, 74 present calls, max score 96.10.

FANTOM5 (CAGE): breadth broad, TPM avg 1.8673 / max 211.6892, expressed in 200 samples.

FANTOM5 promoters (3 alternative TSS)

Promoter ID	TPM avg	Samples expressed
165488	1.5982	167
165489	0.1959	95
165487	0.0732	40

Top tissues by expression

112 total, by Bgee expression score (0-100, higher = more expressed):

Tissue	Anatomy ID	Expression score	Quality
metanephros cortex	UBERON:0010533	96.10	gold quality
adult mammalian kidney	UBERON:0000082	94.45	gold quality
kidney	UBERON:0002113	92.54	gold quality
body of pancreas	UBERON:0001150	90.76	gold quality
cortex of kidney	UBERON:0001225	89.06	gold quality
pancreas	UBERON:0001264	88.94	gold quality
gall bladder	UBERON:0002110	88.19	gold quality
mucosa of transverse colon	UBERON:0004991	87.07	gold quality
islet of Langerhans	UBERON:0000006	85.20	gold quality
right uterine tube	UBERON:0001302	84.16	gold quality
duodenum	UBERON:0002114	83.15	gold quality
right lobe of liver	UBERON:0001114	81.82	gold quality
rectum	UBERON:0001052	81.72	gold quality
liver	UBERON:0002107	80.20	gold quality
male germ line stem cell (sensu Vertebrata) in testis	CL:0000089 ∩ UBERON:0000473	78.28	gold quality
transverse colon	UBERON:0001157	75.48	gold quality
upper lobe of left lung	UBERON:0008952	75.32	gold quality
endometrium	UBERON:0001295	75.06	gold quality
small intestine Peyer’s patch	UBERON:0003454	73.91	gold quality
lung	UBERON:0002048	73.87	gold quality
small intestine	UBERON:0002108	73.79	gold quality
colonic epithelium	UBERON:0000397	72.72	gold quality
right lung	UBERON:0002167	72.28	gold quality
body of stomach	UBERON:0001161	71.85	gold quality
stomach	UBERON:0000945	70.64	gold quality
fallopian tube	UBERON:0003889	69.05	gold quality
testis	UBERON:0000473	66.55	gold quality
left testis	UBERON:0004533	66.30	gold quality
fundus of stomach	UBERON:0001160	65.64	gold quality
right testis	UBERON:0004534	65.52	gold quality

Single-cell (SCXA)

Detected in 3 experiment(s), a significant marker in 3.

Experiment	Marker?	Max mean expression
E-HCAD-10	yes	31.40
E-MTAB-6678	yes	8.11
E-ANND-3	yes	5.07

Regulation

Is transcription factor: yes

Downstream targets (CollecTRI)

71 targets.

Target	Regulation
ABCB6
ABCC2
ACE2
ADAM2
ADCY10
AFM
AFP	Activation
AKR1C4	Activation
ALB	Activation
ALDOB	Repression
B3GALT5	Activation
CA2
CDH1
CDH16
CFTR
CLTRN
CYP2E1
DPP4	Unknown
EGF
EGR2	Activation
FOLR1
FOXA3
FXYD2	Unknown
GJA1
GNAS
GSTA1	Activation
HNF1A
HNF1B
HNF4A	Activation
HP

JASPAR motifs

Motif	Name	Family
MA0153.1	HNF1B	POU domain factors
MA0153.2	HNF1B	POU domain factors

JASPAR matrix evidence (PMIDs): PMID:17916232

Upstream regulators (CollecTRI, top): HNF1B, HNF4A, NR2F1, NR2F2, ONECUT1, PITX2, RBPJ, TBX3

miRNA regulators (miRDB)

72 targeting HNF1B, top 30 by miRDB confidence (max_score; target_count = how many genes the miRNA targets in total — lower means more specific):

miRNA	Max score	Avg score	miRNA target_count
HSA-MIR-4713-3P	100.00	65.92	505
HSA-MIR-4533	100.00	69.48	2758
HSA-MIR-4510	100.00	66.60	2050
HSA-MIR-6127	100.00	66.76	2188
HSA-MIR-6129	100.00	66.46	2080
HSA-MIR-6130	100.00	66.69	2012
HSA-MIR-6133	100.00	66.48	2064
HSA-MIR-520D-5P	99.98	73.34	4883
HSA-MIR-524-5P	99.98	73.43	4882
HSA-MIR-8068	99.98	73.85	2376
HSA-MIR-25-3P	99.98	74.60	1817
HSA-MIR-32-5P	99.98	75.21	1964
HSA-MIR-363-3P	99.98	74.72	1821
HSA-MIR-367-3P	99.98	74.83	1819
HSA-MIR-92A-3P	99.98	75.21	1960
HSA-MIR-92B-3P	99.98	75.25	1955
HSA-MIR-3682-5P	99.93	67.97	1163
HSA-MIR-1297	99.91	73.41	3162
HSA-MIR-30A-3P	99.87	69.74	2928
HSA-MIR-30D-3P	99.87	69.92	2917
HSA-MIR-30E-3P	99.87	69.68	2942
HSA-MIR-26A-5P	99.78	73.52	2303
HSA-MIR-26B-5P	99.78	73.51	2305
HSA-MIR-3156-3P	99.76	66.72	939
HSA-MIR-6752-3P	99.72	66.71	1587
HSA-MIR-4699-3P	99.71	70.15	3142
HSA-MIR-378G	99.71	64.90	1106
HSA-MIR-3202	99.66	67.70	2737
HSA-MIR-545-5P	99.66	70.18	2308
HSA-MIR-24-3P	99.59	69.97	1934

Functional genomics

ClinGen dosage: haploinsufficiency 3 (sufficient evidence), triplosensitivity 0 (no evidence). ClinGen Gene Dosage Map DepMap (CRISPR cell-line fitness): dependent in 12.0% of screened cell lines.

Literature-anchored findings (GeneRIF, showing 40)

• variants of the hepatocyte nuclear factor-1beta (HNF-1beta / TCF2) and the dimerization cofactor of HNF-1 (DcoH/PCBD) genes in relation to type 2 diabetes mellitus and beta-cell function (PMID:11668623)
• an Italian family in which a syndrome of diabetes and severe non-diabetic renal disease is associated with a mutation in the HNF-1Beta gene (PMID:11845237)
• Identification of a gain-of-function mutation in the HNF-1beta gene in a Japanese family with MODY (PMID:11845238)
• Cell-specific involvement of HNF-1beta in alpha(1)-antitrypsin gene expression in human respiratory epithelial cells. (PMID:11880302)
• Solitary functioning kidney and diverse genital tract malformations associated with HNF-1beta mutations (PMID:11918730)
• results confirm that the genetic variations in the HNF-1 beta gene would be a very uncommon cause of progressive nephropathy in patients with type 2 diabetes mellitus (PMID:12012276)
• Renal cysts and diabetes syndrome with nonprogressive liver disorder are linked to mutations of the hepatocyte nuclear factor-1 beta gene. (PMID:12148114)
• Nonsense and missense mutations in the human hepatocyte nuclear factor-1 beta gene (TCF2) and their relation to type 2 diabetes in Japanese. (PMID:12161522)
• Hyperuricemia and young-onset gout are consistent features of the phenotype associated with HNF-1beta mutations, but the mechanism is uncertain. (PMID:12675839)
• Results suggest genetic variants in HNF-1beta are not a common cause of young-onset diabetes or diabetic nephropathy in Chinese, but may modify disease manifestation and progression. (PMID:14583183)
• The reduction of HNF-1 beta expression by RNA interference induced apoptotic cell death in ovarian clear cell carcinoma cells. (PMID:14633622)
• Altered mRNA expression is associated with prostate cancer recurrence. (PMID:15067324)
• diabetes phenotype due to HNF-1beta mutations is similar to that in HNF-1alpha (PMID:15111528)
• HNF1B has several domains involved in nephrogenesis and partially rescues Pax8/lim1-induced kidney malformations. (PMID:15355349)
• HNF-1beta functions as a transcription activator for NNMT gene expression in some papillary thyroid cancer cells (PMID:15486044)
• These results suggest that sucrase-isomaltase transcription might be unchanged or lower in maturity-onset diabetes of the young (MODY) type 3, but greater in MODY5. (PMID:15522234)
• the hepatocyte nuclear factor-1beta (HNF-1beta) C-terminal domain has a role in Pkhd1 (ARPKD) gene transcription and renal cystogenesis (PMID:15647252)
• HNF-1beta gene mutations may be associated with nondiabetic renal dysfunction and diabetes in Chinese, but they are responsible for only a small percentage of early onset or multiple affected diabetes pedigrees including MODY. (PMID:15660195)
• transcription factors hepatic nuclear factor 1 (HNF1)alpha and beta play an important part in the regulation of the ACAT2 promoter (PMID:15961790)
• In 40 unrelated patients presenting with MODY5 phenotype, TCF2 was screened for mutations by sequencing. (PMID:16249435)
• Results indicate that HNF-1beta is an excellent molecular marker for ovarian clear cell tumors. (PMID:16258507)
• we propose that HNF1beta overexpression in the ovarian cancer participates in the altered expression pattern (PMID:16297991)
• Subjects with HNF-1beta mutations have reduced insulin sensitivity of endogenous glucose production but normal peripheral insulin sensitivity in contrast to patients with HNF-1alpha mutations and normal controls. (PMID:16443774)
• results suggest that TCF2 is involved in the development of ovarian cancers and may represent a useful target for their detection and treatment (PMID:16479257)
• No mutation was identified in the HNF-1beta genes in Japanese patients with juvenile-onset (before 18 years of age) non-obese diabetes mellitus. (PMID:16562587)
• depsipeptide represses NNMT and HNF-1beta gene expression in some papillary thyroid cancer cells (PMID:16676400)
• both wild HNF-1alpha and wild HNF-1beta have a stimulatory effect on dipeptidylpeptidase IV gene expression, but that mutant HNF-1alpha and mutant HNF-1beta attenuate the stimulatory effect (PMID:16781669)
• These results indicate that the tissue-specific expression of hOAT3 might be regulated by the concerted effect of genetic (HNF1alpha and HNF1beta) and epigenetic (DNA methylation) factors. (PMID:16793932)
• Evidence of differential gene-dosage requirements for HNF1beta in normal human kidney and pancreas differentiation. (PMID:16801329)
• Mutations in TCF2 is associated with renal hypodysplasia (PMID:16971658)
• a key role for HNF-1beta in early pancreatic progenitor cells in man (PMID:17116179)
• Heterozygous deletion of the TCF2 gene is an important cause of fetal hyperechogenic kidneys in this study and showed to be linked with early disease expression. (PMID:17267738)
• A study evaluating the extent to which common variation in the six known maturity-onset diabetes of the young (MODY) genes, which cause a monogenic form of type 2 diabetes, is associated with type 2 diabetes is presented. (PMID:17327436)
• TCF2 is a biological tumor marker for epithelial ovarian cancers. (PMID:17503391)
• Two variants on chromosome 17q contribute to the risk of prostate cancer in 4 populations, one of the variants is in TCF2 confers protection against type 2 diabetes. (PMID:17603485)
• R276X functions in a negative manner in regard to metabolic responses of insulin secretion in beta cells. (PMID:17878605)
• no direct relationship between horseshoe kidney in TS and mutation or polymorphism of HNF-1beta gene, but we speculate that target gene(s) of HNF-1beta, likely mapped on the X chromosome, is/are responsible of the horseshoe kidney formation in TS. (PMID:17922147)
• Findings implicate HNF1beta as a regulator of pancreas organogenesis and differentiation. [REVIEW] (PMID:17923767)
• help us to understand the structural basis of promoter recognition by these atypical POU transcription factors and the site-specific functional disruption by disease-causing mutations (PMID:17924661)
• the up-regulation of TCF11/MafG binding could be suppressed by overexpression of the TGF-beta inhibitor Smad7, and a small interfering RNA to TCF11 blocked the suppression of iNOS by TGF-beta. (PMID:17928287)

Cross-species orthologs

4 orthologs

Organism	Symbol	Gene ID
danio_rerio	hnf1ba	ENSDARG00000006615
danio_rerio	hnf1bb	ENSDARG00000022295
mus_musculus	Hnf1b	ENSMUSG00000020679
rattus_norvegicus	Hnf1b	ENSRNOG00000002598

Paralogs (1): HNF1A (ENSG00000135100)

Protein

Protein identifiers

Hepatocyte nuclear factor 1-beta — P35680 (reviewed: P35680)

Alternative names: Homeoprotein LFB3, Transcription factor 2, Variant hepatic nuclear factor 1

All UniProt accessions (5): P35680, A0A087WZC2, A0A0C4DGS8, E0YMJ6, Q6FHW6

UniProt curated annotations — full annotation on UniProt →

Function. Transcription factor that binds to the inverted palindrome 5’-GTTAATNATTAAC-3’. Binds to the FPC element in the cAMP regulatory unit of the PLAU gene. Transcriptional activity is increased by coactivator PCBD1.

Subunit / interactions. Binds DNA as a dimer. Can form homodimer or heterodimer with HNF1-alpha. Interacts (via HNF-p1 domain) with PCBD1; the interaction increases its transactivation activity.

Subcellular location. Nucleus.

Disease relevance. Renal cysts and diabetes syndrome (RCAD) [MIM:137920] An autosomal dominant disorder comprising non-diabetic renal disease resulting from abnormal renal development, and diabetes, which in some cases occurs earlier than age 25 years and is thus consistent with a diagnosis of maturity-onset diabetes of the young (MODY5). The renal disease is highly variable and includes renal cysts, glomerular tufts, aberrant nephrogenesis, primitive tubules, irregular collecting systems, oligomeganephronia, enlarged renal pelves, abnormal calyces, small kidney, single kidney, horseshoe kidney, and hyperuricemic nephropathy. Affected individuals may also have abnormalities of the genital tract. The disease is caused by variants affecting the gene represented in this entry. Type 2 diabetes mellitus (T2D) [MIM:125853] A multifactorial disorder of glucose homeostasis caused by a lack of sensitivity to insulin. Affected individuals usually have an obese body habitus and manifestations of a metabolic syndrome characterized by diabetes, insulin resistance, hypertension and hypertriglyceridemia. The disease results in long-term complications that affect the eyes, kidneys, nerves, and blood vessels. Disease susceptibility may be associated with variants affecting the gene represented in this entry. Prostate cancer, hereditary, 11 (HPC11) [MIM:611955] A condition associated with familial predisposition to cancer of the prostate. Most prostate cancers are adenocarcinomas that develop in the acini of the prostatic ducts. Other rare histopathologic types of prostate cancer that occur in approximately 5% of patients include small cell carcinoma, mucinous carcinoma, prostatic ductal carcinoma, transitional cell carcinoma, squamous cell carcinoma, basal cell carcinoma, adenoid cystic carcinoma (basaloid), signet-ring cell carcinoma and neuroendocrine carcinoma. Disease susceptibility is associated with variants affecting the gene represented in this entry.

Similarity. Belongs to the HNF1 homeobox family.

Isoforms (4)

UniProt ID	Names	Canonical?
P35680-1	A	yes
P35680-2	B
P35680-3	C
P35680-4	4

RefSeq proteins (4): NP_000449, NP_001159395, NP_001291215, NP_001398029 (=MANE)

Domains & families (InterPro)

ID	Name	Type
IPR001356	HD	Domain
IPR006897	HNF1b_C	Domain
IPR006899	HNF-1_N	Domain
IPR009057	Homeodomain-like_sf	Homologous_superfamily
IPR010982	Lambda_DNA-bd_dom_sf	Homologous_superfamily
IPR023219	HNF1_dimer_N_dom_sf	Homologous_superfamily
IPR039066	HNF-1	Family
IPR044866	HNF_P1	Domain
IPR044869	HNF-1_POU	Domain

Pfam: PF04812, PF04814

UniProt features (57 total): sequence variant 25, helix 10, splice variant 6, modified residue 4, region of interest 3, domain 2, mutagenesis site 2, chain 1, DNA-binding region 1, turn 1, strand 1, compositionally biased region 1

Structure

Experimental structures (PDB)

3 structures.

PDB	Method	Resolution (Å)
5K9S	X-RAY DIFFRACTION	2.4
2H8R	X-RAY DIFFRACTION	3.2
2DA6	SOLUTION NMR

Predicted structure (AlphaFold)

Model	pLDDT	Fraction very-high
AF-P35680-F1	61.91	0.29

Functional residue map

Curated UniProt residues grouped by drug-discovery relevance — catalytic, ligand-binding, modification, and mutation-validated positions. Source: UniProtKB sequence features.

Post-translational modifications (4): 75, 80, 49, 52

Mutagenesis-validated functional residues (2):

Position	Phenotype
1–32	loss of interaction with pcbd1. loss of pcbd1 recruitment to the nucleus.
253	no impact on interaction with pcbd1. reduced pcbd1 recruitment to the nucleus. reduced coactivation by pcbd1.

Function

Pathways and Gene Ontology

Reactome pathways

6 pathways

ID	Pathway
R-HSA-210744	Regulation of gene expression in late stage (branching morphogenesis) pancreatic bud precursor cells
R-HSA-210747	Regulation of gene expression in early pancreatic precursor cells
R-HSA-9831926	Nephron development
R-HSA-9925561	Developmental Lineage of Pancreatic Acinar Cells
R-HSA-9925563	Developmental Lineage of Pancreatic Ductal Cells
R-HSA-9937080	Developmental Lineage of Multipotent Pancreatic Progenitor Cells

MSigDB gene sets: 495 (showing top): GOBP_MORPHOGENESIS_OF_AN_EPITHELIUM, GOBP_HINDBRAIN_DEVELOPMENT, GOBP_HEPATICOBILIARY_SYSTEM_DEVELOPMENT, GOBP_EMBRYO_DEVELOPMENT_ENDING_IN_BIRTH_OR_EGG_HATCHING, GOBP_EPITHELIUM_DEVELOPMENT, GOBP_SOMATIC_STEM_CELL_POPULATION_MAINTENANCE, BUYTAERT_PHOTODYNAMIC_THERAPY_STRESS_DN, BENPORATH_ES_WITH_H3K27ME3, GRUETZMANN_PANCREATIC_CANCER_DN, GOBP_METANEPHROS_DEVELOPMENT, SWEET_KRAS_ONCOGENIC_SIGNATURE, PID_HNF3B_PATHWAY, MIDORIKAWA_AMPLIFIED_IN_LIVER_CANCER, GOBP_FORMATION_OF_PRIMARY_GERM_LAYER, GOBP_REGULATION_OF_DEVELOPMENTAL_GROWTH

GO Biological Process (44): negative regulation of transcription by RNA polymerase II (GO:0000122), endodermal cell fate specification (GO:0001714), kidney development (GO:0001822), inner cell mass cell differentiation (GO:0001826), regulation of transcription by RNA polymerase II (GO:0006357), Notch signaling pathway (GO:0007219), response to glucose (GO:0009749), anterior/posterior pattern specification (GO:0009952), gene expression (GO:0010467), positive regulation of gene expression (GO:0010628), insulin secretion (GO:0030073), regulation of Wnt signaling pathway (GO:0030111), hindbrain development (GO:0030902), pancreas development (GO:0031016), endocrine pancreas development (GO:0031018), regulation of pronephros size (GO:0035565), pronephric nephron tubule development (GO:0039020), positive regulation of DNA-templated transcription (GO:0045893), embryonic digestive tract morphogenesis (GO:0048557), branching morphogenesis of an epithelial tube (GO:0048754), pronephros development (GO:0048793), genitalia development (GO:0048806), epithelial cell proliferation (GO:0050673), positive regulation of transcription initiation by RNA polymerase II (GO:0060261), ureteric bud elongation (GO:0060677), hepatoblast differentiation (GO:0061017), obsolete negative regulation of mesenchymal cell apoptotic process involved in mesonephric nephron morphogenesis (GO:0061296), regulation of branch elongation involved in ureteric bud branching (GO:0072095), mesonephric duct formation (GO:0072181), mesenchymal cell apoptotic process involved in metanephros development (GO:1900200), negative regulation of mesenchymal cell apoptotic process involved in metanephros development (GO:1900212), liver development (GO:0001889), apoptotic process (GO:0006915), endoderm development (GO:0007492), negative regulation of apoptotic process (GO:0043066), positive regulation of transcription by RNA polymerase II (GO:0045944), animal organ development (GO:0048513), system development (GO:0048731), epithelium development (GO:0060429), kidney morphogenesis (GO:0060993)

GO Molecular Function (11): RNA polymerase II cis-regulatory region sequence-specific DNA binding (GO:0000978), DNA-binding transcription factor activity, RNA polymerase II-specific (GO:0000981), transcription coregulator binding (GO:0001221), DNA binding (GO:0003677), DNA-binding transcription factor activity (GO:0003700), identical protein binding (GO:0042802), protein homodimerization activity (GO:0042803), promoter-specific chromatin binding (GO:1990841), transcription cis-regulatory region binding (GO:0000976), cis-regulatory region sequence-specific DNA binding (GO:0000987), protein binding (GO:0005515)

GO Cellular Component (5): chromatin (GO:0000785), nucleus (GO:0005634), nucleoplasm (GO:0005654), transcription regulator complex (GO:0005667), cytosol (GO:0005829)

Reactome top-level categories

Rollup of top-3 pathways:

Category	Pathways
Developmental Cell Lineages of the Exocrine Pancreas	3
Regulation of beta-cell development	2
Kidney development	1

GO top-level categories

Rollup of top GO terms by namespace:

Category	Terms
regulation of DNA-templated transcription	3
cellular anatomical structure	3
regulation of transcription by RNA polymerase II	2
transcription by RNA polymerase II	2
animal organ development	2
anatomical structure development	2
RNA polymerase II transcription regulatory region sequence-specific DNA binding	2
transcription cis-regulatory region binding	2
negative regulation of DNA-templated transcription	1
cell fate specification	1
endodermal cell fate commitment	1
renal system development	1
blastocyst formation	1
cell differentiation	1
cell surface receptor signaling pathway	1
response to hexose	1
regionalization	1
macromolecule biosynthetic process	1
gene expression	1
regulation of gene expression	1
positive regulation of macromolecule biosynthetic process	1
protein secretion	1
peptide hormone secretion	1
regulation of signal transduction	1
Wnt signaling pathway	1
brain development	1
pancreas development	1
endocrine system development	1
regulation of kidney size	1
pronephros morphogenesis	1
pronephric nephron development	1
nephron tubule development	1
DNA-templated transcription	1
positive regulation of RNA biosynthetic process	1
digestive tract morphogenesis	1
embryonic organ morphogenesis	1
embryonic digestive tract development	1
tube morphogenesis	1
epithelial tube morphogenesis	1
morphogenesis of a branching epithelium	1

Protein interactions and networks

STRING

1412 interactions, top by confidence (×1000):

Protein A	Protein B	Partner UniProt	Score
HNF1B	ONECUT1	Q9UBC0	827
HNF1B	PKHD1	P08F94	827
HNF1B	HNF4A	P41235	804
HNF1B	UMOD	P07911	802
HNF1B	PDX1	P52945	798
HNF1B	HNF1A	P20823	786
HNF1B	NEUROD1	Q13562	762
HNF1B	GCK	P35557	755
HNF1B	HHEX	Q03014	716
HNF1B	NEUROG3	Q9Y4Z2	714
HNF1B	CDKAL1	Q5VV42	706
HNF1B	PAX4	O43316	705
HNF1B	NAPSA	O96009	702
HNF1B	JAZF1	Q86VZ6	672
HNF1B	NR2F2	P24468	656

IntAct

45 interactions, top by confidence:

A	B	Type	Score
HNF1B	PCBD1	psi-mi:“MI:0915”(physical association)	0.810
PCBD1	HNF1B	psi-mi:“MI:0915”(physical association)	0.810
HNF1B	VAC14	psi-mi:“MI:0915”(physical association)	0.560
HNF1B	USP54	psi-mi:“MI:0915”(physical association)	0.560
HNF1B	POU6F2	psi-mi:“MI:0915”(physical association)	0.560
CRX	HNF1B	psi-mi:“MI:0915”(physical association)	0.560
HNF1B	C10orf55	psi-mi:“MI:0915”(physical association)	0.560
HNF1B	KRTAP26-1	psi-mi:“MI:0915”(physical association)	0.560
HNF1B	BPIFA1	psi-mi:“MI:0915”(physical association)	0.560
HNF1B	OXER1	psi-mi:“MI:0915”(physical association)	0.560
HNF1B	MLLT10	psi-mi:“MI:0915”(physical association)	0.560
OTX2	HNF1B	psi-mi:“MI:0915”(physical association)	0.560
HNF1B	UFSP1	psi-mi:“MI:0915”(physical association)	0.560
HNF1B	HNF1A	psi-mi:“MI:0915”(physical association)	0.560
HNF1B	GFPT2	psi-mi:“MI:0914”(association)	0.350
PCBD1	UBB	psi-mi:“MI:0914”(association)	0.350
PCBD2	DBT	psi-mi:“MI:0914”(association)	0.350
HNF1B	BCL9	psi-mi:“MI:2364”(proximity)	0.270
HNF1B	VAC14	psi-mi:“MI:0915”(physical association)	0.000
HNF1B	USP54	psi-mi:“MI:0915”(physical association)	0.000
HNF1B	PCBD1	psi-mi:“MI:0915”(physical association)	0.000
HNF1B	POU6F2	psi-mi:“MI:0915”(physical association)	0.000
HNF1B	CRX	psi-mi:“MI:0915”(physical association)	0.000
HNF1B	OTX2	psi-mi:“MI:0915”(physical association)	0.000
HNF1B	UFSP1	psi-mi:“MI:0915”(physical association)	0.000
HNF1B	C10orf55	psi-mi:“MI:0915”(physical association)	0.000
HNF1B	KRTAP26-1	psi-mi:“MI:0915”(physical association)	0.000

BioGRID (203): CSNK1A1 (Affinity Capture-MS), HNF1B (Reconstituted Complex), HNF1B (Reconstituted Complex), HNF1B (Affinity Capture-MS), PCBD1 (Affinity Capture-MS), PARP1 (Affinity Capture-MS), PCBP1 (Affinity Capture-MS), HSPA5 (Affinity Capture-MS), CLIC1 (Affinity Capture-MS), EIF4A3 (Affinity Capture-MS), HNF1B (Affinity Capture-MS), RUVBL1 (Affinity Capture-MS), EWSR1 (Affinity Capture-MS), RPL27A (Affinity Capture-MS), XRCC6 (Affinity Capture-MS)

ESM2 similar proteins: A0A0G2JTZ2, A2BEA6, A4IFD2, B1H349, B3DM43, B3DM47, F1M8W4, O15409, O75030, P0CF24, P23899, P27889, P35680, P35710, P35711, P35712, P40645, P40647, P58463, P70062, P70063, P70064, Q23045, Q2LE08, Q4VYR7, Q4VYS1, Q58NQ4, Q5QL03, Q5RCU4, Q5RER5, Q5W1J5, Q6GL68, Q800Q5, Q86MD3, Q8HZ00, Q8MJ97, Q8MJ98, Q8MJ99, Q8MJA0, Q8STF6

Diamond homologs: A1L1N5, P15257, P20823, P22361, P23899, P27889, P35680, Q03365, Q05041, Q5RER5, Q8UW00, Q90867, Q91474, Q91739, Q91910

SIGNOR signaling

9 interactions.

A	Effect	B	Mechanism
HNF1B	“up-regulates quantity by expression”	AKR1C4	“transcriptional regulation”
HNF1B	“up-regulates activity”	ATF1	binding
HNF1B	“up-regulates activity”	CREB1	binding
HNF1B	“up-regulates quantity by expression”	FXYD2	“transcriptional regulation”
HNF1B	“up-regulates quantity by expression”	ALB	“transcriptional regulation”
HNF1B	“up-regulates quantity by expression”	UMOD	“transcriptional regulation”
HNF1B	“up-regulates quantity by expression”	AFP	“transcriptional regulation”
PCBD1	“up-regulates activity”	HNF1B	binding

Disease & clinical

Clinical variants and AI predictions

ClinVar

890 variants total. Per-class counts are floors (≥ shown; pagination cap):

Classification	Count (floor)
Pathogenic	155
Likely pathogenic	82
Uncertain significance	265
Likely benign	166
Benign	47

Top pathogenic / likely-pathogenic (30)

Variant ID	HGVS	Classification
12635	NM_000458.4(HNF1B):c.529C>T (p.Arg177Ter)	Pathogenic
12638	NM_000458.4(HNF1B):c.301G>T (p.Glu101Ter)	Pathogenic
12639	HNF1B, 1-BP DEL	Pathogenic
12640	NM_000458.4(HNF1B):c.826C>T (p.Arg276Ter)	Pathogenic
12642	NM_000458.4(HNF1B):c.1055dup (p.Tyr352Ter)	Pathogenic
12644	NM_000458.4(HNF1B):c.544+1G>T	Pathogenic
12645	NM_000458.4(HNF1B):c.443C>G (p.Ser148Trp)	Pathogenic
12646	HNF1B, EX5DUP	Pathogenic
12648	NM_000458.4(HNF1B):c.46del (p.Leu16fs)	Pathogenic
1338597	NM_000458.4(HNF1B):c.807_809+3del	Pathogenic
1344654	NM_000458.4(HNF1B):c.1333_1334del (p.Ala445fs)	Pathogenic
1359835	NM_000458.4(HNF1B):c.1063C>T (p.Gln355Ter)	Pathogenic
1457998	NM_000458.4(HNF1B):c.815_825del (p.Glu272fs)	Pathogenic
1706960	NM_000458.4(HNF1B):c.344+2_344+5del	Pathogenic
1802162	NM_000458.4(HNF1B):c.221T>G (p.Leu74Trp)	Pathogenic
1992355	NM_000458.4(HNF1B):c.280G>T (p.Glu94Ter)	Pathogenic
2012853	NM_000458.4(HNF1B):c.344+2T>G	Pathogenic
2022853	NM_000458.4(HNF1B):c.783_786del (p.Glu262fs)	Pathogenic
2105603	NM_000458.4(HNF1B):c.1131_1134dup (p.Ser379fs)	Pathogenic
2163399	NM_000458.4(HNF1B):c.25C>T (p.Gln9Ter)	Pathogenic
224334	NM_000458.4(HNF1B):c.1132dup (p.Gln378fs)	Pathogenic
2573411	NC_000017.10:g.(?36046433)(36105070_?)del	Pathogenic
2582407	NM_000458.4(HNF1B):c.515del (p.Tyr172fs)	Pathogenic
3026928	NM_000458.4(HNF1B):c.1042del (p.Ser348fs)	Pathogenic
3061133	NM_000458.4(HNF1B):c.785_786del (p.Glu262fs)	Pathogenic
3234111	NM_000458.4(HNF1B):c.1397_1404del (p.Leu466fs)	Pathogenic
3235201	NM_000458.4(HNF1B):c.92_99dup (p.Leu34fs)	Pathogenic
3236049	NM_000458.4(HNF1B):c.827_837del (p.Arg276fs)	Pathogenic
3254996	NM_000458.4(HNF1B):c.370del (p.Lys123_Met124insTer)	Pathogenic
3255163	NM_000458.4(HNF1B):c.1067dup (p.Asn357fs)	Pathogenic

SpliceAI

1371 predictions. Top by Δscore:

Variant	Effect	Δscore
17:37687393:C:CC	acceptor_gain	1.0000
17:37699072:TTA:T	donor_loss	1.0000
17:37699073:TAC:T	donor_loss	1.0000
17:37699074:AC:A	donor_loss	1.0000
17:37699075:CCTG:C	donor_loss	1.0000
17:37699192:ACA:A	acceptor_gain	1.0000
17:37699193:CA:C	acceptor_gain	1.0000
17:37699193:CAC:C	acceptor_gain	1.0000
17:37699195:C:CC	acceptor_gain	1.0000
17:37700977:CCTTA:C	donor_loss	1.0000
17:37700978:CTTAC:C	donor_loss	1.0000
17:37700979:TTACT:T	donor_loss	1.0000
17:37700980:TA:T	donor_loss	1.0000
17:37700981:A:AC	donor_gain	1.0000
17:37700981:ACT:A	donor_loss	1.0000
17:37700982:C:CC	donor_gain	1.0000
17:37710497:ACT:A	donor_loss	1.0000
17:37710498:CTC:C	donor_loss	1.0000
17:37710499:TCAC:T	donor_loss	1.0000
17:37710500:CA:C	donor_loss	1.0000
17:37710502:C:CT	donor_loss	1.0000
17:37733552:GTTA:G	donor_loss	1.0000
17:37733554:TA:T	donor_loss	1.0000
17:37733590:T:TA	donor_gain	1.0000
17:37739640:C:CC	acceptor_gain	1.0000
17:37744535:GCCTA:G	donor_loss	1.0000
17:37744536:CCTAC:C	donor_loss	1.0000
17:37744537:CTACC:C	donor_loss	1.0000
17:37744538:TA:T	donor_loss	1.0000
17:37744539:ACC:A	donor_loss	1.0000

AlphaMissense

0 scored. Top likely-pathogenic:

dbSNP variants (sampled 300 via entrez): RS1000001916 (17:37736355 TGTG>T), RS1000061004 (17:37741649 AC>A), RS1000071885 (17:37725773 C>T), RS1000079082 (17:37693304 T>C), RS1000091664 (17:37741305 A>C), RS1000102235 (17:37734424 A>G), RS1000124219 (17:37712130 C>A), RS1000152567 (17:37693602 C>T), RS1000185843 (17:37687461 T>G), RS1000252004 (17:37710353 A>C,G), RS1000259172 (17:37728602 G>A,T), RS1000367424 (17:37722172 T>C), RS1000518126 (17:37721597 G>A), RS1000571783 (17:37721328 G>A), RS1000667357 (17:37687767 G>A)

Disease associations

OMIM: gene MIM:189907 | disease phenotypes: MIM:125853, MIM:137920, MIM:144700, MIM:125850, MIM:606391, MIM:167000, MIM:174050, MIM:610805, MIM:614227

GenCC curated gene-disease

Disease	Classification	Inheritance
renal cysts and diabetes syndrome	Definitive	Autosomal dominant
transient neonatal diabetes mellitus	Strong	Autosomal dominant
permanent neonatal diabetes mellitus	Strong	Autosomal dominant
diabetes mellitus, noninsulin-dependent	Strong	Autosomal dominant
medullary sponge kidney	Supportive	Autosomal dominant
renal hypomagnesemia 2	Supportive	Autosomal dominant
renal dysplasia, unilateral	Supportive	Autosomal dominant
renal dysplasia, bilateral	Supportive	Autosomal dominant
unilateral multicystic dysplastic kidney	Supportive	Autosomal dominant

ClinGen Gene-Disease Validity (1)

Expert-panel classifications — Definitive > Strong > Moderate > Limited > Disputed > Refuted.

Disease	Classification	Inheritance
renal cysts and diabetes syndrome	Definitive	AD

Mondo (21): type 2 diabetes mellitus (MONDO:0005148), renal cysts and diabetes syndrome (MONDO:0007669), nonpapillary renal cell carcinoma (MONDO:0007763), autosomal dominant medullary cystic kidney disease with or without hyperuricemia (MONDO:0008264), maturity-onset diabetes of the young (MONDO:0018911), ovarian cancer (MONDO:0008170), chromophobe renal cell carcinoma (MONDO:0017885), autosomal dominant polycystic liver disease (MONDO:0000447), monogenic diabetes (MONDO:0015967), congenital anomaly of kidney and urinary tract (MONDO:0019719), diabetes mellitus (MONDO:0005015), cystic kidney disease (MONDO:0002473), hyperuricemic nephropathy, familial juvenile type 3 (MONDO:0013643), transient neonatal diabetes mellitus (MONDO:0020525), permanent neonatal diabetes mellitus (MONDO:0100164)

Orphanet (9): Hereditary clear cell renal cell carcinoma (Orphanet:422526), HNF1B-related autosomal dominant tubulointerstitial kidney disease (Orphanet:93111), Autosomal dominant tubulointerstitial kidney disease (Orphanet:34149), MODY (Orphanet:552), Rare ovarian cancer (Orphanet:213500), Chromophobe renal cell carcinoma (Orphanet:319303), Rare genetic diabetes mellitus (Orphanet:183625), Isolated polycystic liver disease (Orphanet:2924), Renal or urinary tract malformation (Orphanet:93545)

HPO phenotypes

90 total (30 of 90 shown, HPO-id order):

HPO	Term
HP:0000003	Multicystic kidney dysplasia
HP:0000006	Autosomal dominant inheritance
HP:0000013	Hypoplasia of the uterus
HP:0000027	Azoospermia
HP:0000028	Cryptorchidism
HP:0000047	Hypospadias
HP:0000049	Shawl scrotum
HP:0000070	Ureterocele
HP:0000074	Ureteropelvic junction obstruction
HP:0000077	Abnormality of the kidney
HP:0000078	Abnormality of the genital system
HP:0000083	Renal insufficiency
HP:0000085	Horseshoe kidney
HP:0000086	Ectopic kidney
HP:0000089	Renal hypoplasia
HP:0000093	Proteinuria
HP:0000104	Renal agenesis
HP:0000107	Renal cyst
HP:0000110	Renal dysplasia
HP:0000122	Unilateral renal agenesis
HP:0000239	Large fontanelles
HP:0000303	Mandibular prognathia
HP:0000365	Hearing impairment
HP:0000470	Short neck
HP:0000717	Autism
HP:0000772	Abnormal rib morphology
HP:0000787	Nephrolithiasis
HP:0000790	Hematuria
HP:0000813	Bicornuate uterus
HP:0000819	Diabetes mellitus

GWAS associations

60 associations (top):

Study	Trait	p-value
GCST000050_2	Prostate cancer	1.000000e-11
GCST000152_11	Prostate cancer	9.000000e-12
GCST000154_7	Prostate cancer	1.000000e-09
GCST000712_12	Type 2 diabetes	2.000000e-06
GCST000750_9	Prostate cancer	1.000000e-12
GCST000919_7	Serum prostate-specific antigen levels	6.000000e-11
GCST001040_2	Endometrial cancer	7.000000e-10
GCST001147_2	Prostate cancer	2.000000e-06
GCST001666_6	Type 2 diabetes	2.000000e-11
GCST001941_16	Ovarian cancer	8.000000e-10
GCST002128_12	Type 2 diabetes	4.000000e-06
GCST002352_49	Type 2 diabetes	9.000000e-10
GCST002413_5	Prostate cancer (early onset)	1.000000e-06
GCST002592_2	Neuritic plaque	1.000000e-06
GCST002748_9	Epithelial ovarian cancer	2.000000e-08
GCST002855_1	Testicular germ cell tumor	1.000000e-09
GCST002890_4	Prostate cancer	8.000000e-29
GCST002960_9	Frontotemporal dementia	4.000000e-06
GCST003148_14	Prostate cancer	6.000000e-08
GCST003400_10	Type 2 diabetes	4.000000e-15
GCST003436_4	Endometrial cancer	1.000000e-08
GCST003524_6	Endometrial cancer	3.000000e-19
GCST003525_5	Endometrial endometrioid carcinoma	7.000000e-17
GCST003586_6	Prostate cancer	3.000000e-10
GCST003587_13	Cancer	1.000000e-09
GCST003588_20	Cancer (pleiotropy)	7.000000e-28
GCST004093_10	Prostate-specific antigen levels	6.000000e-20
GCST004635_35	Testicular germ cell tumor	3.000000e-20
GCST004713_35	Testicular germ cell tumor	1.000000e-14
GCST004894_80	Type 2 diabetes	3.000000e-15

EFO canonical traits (11, from GWAS)

EFO ID	Trait name
EFO:0004230	endometrial neoplasm
EFO:0006798	neuritic plaque measurement
EFO:1001514	endometrial endometrioid carcinoma
EFO:1001515	ovarian endometrioid carcinoma
EFO:1001516	ovarian serous carcinoma
EFO:0004340	body mass index
EFO:0008008	lower urinary tract symptom
EFO:0009924	Drugs used in diabetes use measurement
EFO:0004458	C-reactive protein measurement
EFO:0004736	aspartate aminotransferase measurement
EFO:0004532	serum gamma-glutamyl transferase measurement

MeSH disease descriptors (11)

Descriptor	Name	Tree numbers
D003920	Diabetes Mellitus	C18.452.394.750; C19.246
D003924	Diabetes Mellitus, Type 2	C18.452.394.750.149; C19.246.300
D052177	Kidney Diseases, Cystic	C12.050.351.968.419.403; C12.200.777.419.403; C12.950.419.403
D007691	Medullary Sponge Kidney	C12.050.351.968.419.403.500; C12.200.777.419.403.500; C12.950.419.403.500
D010051	Ovarian Neoplasms	C04.588.322.455; C12.050.351.500.056.630.705; C12.050.351.937.418.685; C12.100.250.056.630.705; C12.900.418.685; C19.344.410; C19.391.630.705
C566906	Cakut (supp.)
C563425	Diabetes Mellitus, Permanent Neonatal (supp.)
C537152	Hypomagnesemia 2, renal (supp.)
C562772	Mason-Type Diabetes (supp.)
C536137	Medullary cystic kidney disease 1 (supp.)
C535520	Renal cysts and diabetes syndrome (supp.)

Drugs & pharmacology

Drug and pharmacology data

Is drug target: no

PharmGKB: 1 entry (VIP=true, CPIC=false)

PharmGKB variants

1 variants.

Variant	Genes	Level	Score	#Clin annots	Drugs
rs11868513	HNF1B		0.00	0

CTD chemical–gene interactions

44 total (human), top 30 by PubMed support.

Chemical	Actions (top 5)	PubMed papers
(+)-JQ1 compound	decreases expression	2
Benzo(a)pyrene	increases methylation, affects methylation, decreases expression	2
Tetrachlorodibenzodioxin	increases expression	2
Valproic Acid	affects expression, decreases expression, decreases methylation	2
aristolochic acid I	decreases expression	1
dicrotophos	increases expression	1
bisphenol A	affects reaction, affects binding, decreases reaction, decreases expression, increases uptake (+1 more)	1
trichostatin A	affects expression, decreases reaction	1
arsenite	increases methylation	1
sodium arsenite	increases expression	1
butyraldehyde	decreases expression	1
S-(1,2-dichlorovinyl)cysteine	affects cotreatment, increases expression	1
2-amino-1-methyl-6-phenylimidazo(4,5-b)pyridine	decreases expression	1
perfluorooctane sulfonic acid	decreases expression	1
CGP 52608	affects binding, increases reaction	1
2-palmitoylglycerol	increases expression	1
clothianidin	increases expression	1
abrine	decreases expression	1
enzalutamide	affects expression	1
Resveratrol	decreases expression	1
Fulvestrant	decreases expression	1
Leflunomide	increases expression	1
Microplastics	decreases expression, increases abundance	1
Acetaminophen	decreases expression	1
Dexamethasone	affects binding, affects cotreatment, increases reaction	1
Diazinon	increases methylation	1
Dimethyl Sulfoxide	increases expression	1
Fluorocarbons	affects cotreatment, increases expression	1
Folic Acid	increases methylation, increases expression	1
Glucose	decreases reaction, increases uptake	1

Cellosaurus cell lines

10 cell lines: 5 cancer cell line, 4 embryonic stem cell, 1 induced pluripotent stem cell

First 10 cell lines (id-ordered, not curated):

Cellosaurus	Name	Category	Sex
CVCL_A0ZS	PLAFMCi005-A	Induced pluripotent stem cell	Male
CVCL_A7C5	SEES3-1V human TCF2, clone1	Embryonic stem cell	Male
CVCL_A7C6	SEES3-1V human TCF2, clone2	Embryonic stem cell	Male
CVCL_A7C7	SEES3-1V human TCF2, clone3	Embryonic stem cell	Male
CVCL_B7XL	Abcam Raji HNF1B KO	Cancer cell line	Male
CVCL_B9YA	Abcam THP-1 HNF1B KO	Cancer cell line	Male
CVCL_C7A3	Abcam PC-3 HNF1B KO	Cancer cell line	Male
CVCL_D3XF	HepG2/8F_HS	Cancer cell line	Male
CVCL_D8MH	Ubigene HCT 116 HNF1B KO	Cancer cell line	Male
CVCL_E3RS	UMANe002-A-4	Embryonic stem cell	Male

Clinical trials (associated diseases)

306 trials via MONDO — disease-level, not drug-specific.

Trial	Phase	Status	Title
NCT02624817	PHASE4	COMPLETED	Long-Term Sulfonylurea Response in KCNJ11 Neonatal Diabetes
NCT02624830	PHASE4	UNKNOWN	Long-Term Sulfonylurea Response in ABCC8 Neonatal Diabetes (SuResponsSUR)
NCT00006163	PHASE4	COMPLETED	Computer-assisted Diabetes Self-management Interventions
NCT00036504	PHASE4	COMPLETED	Efficacy and Safety of Twice-Daily Insulin Lispro Low Mixture Compared to a Once-Daily Long Acting Insulin Comparator in Patients Who Have Been Using One or More Oral Antihyperglycemic Agents Without Insulin
NCT00044460	PHASE4	COMPLETED	Efficacy and Safety In Poorly Controlled Type 2 Diabetics
NCT00095446	PHASE4	COMPLETED	NovoLog Observation Trial in Subjects With Type 1 and Type 2 Diabetes
NCT00101751	PHASE4	COMPLETED	INITIATE Plus (INITiation of Insulin to Reach A1c TargEt) Study
NCT00110370	PHASE4	COMPLETED	Comparing Pre-Mixed Insulin With Insulin Glargine Combined With Rapid-Acting Insulin in Patients With Type 2 Diabetes
NCT00110448	PHASE4	COMPLETED	Japanese Primary Prevention of Atherosclerosis With Aspirin for Diabetes (JPAD) Trial
NCT00118950	PHASE4	COMPLETED	Effect of Metformin Versus Repaglinide Treatment in Non-Obese Type 2 Diabetic Patients Uncontrolled by Diet
NCT00118963	PHASE4	COMPLETED	Effect of Repaglinide Versus Metformin Treatment in Non-Obese Patients With Type-2-Diabetes
NCT00121966	PHASE4	COMPLETED	South Danish Diabetes Study: Evaluation of the Antidiabetic Treatment of Type 2 Diabetes Mellitus
NCT00123604	PHASE4	COMPLETED	Vascular Effects of Carvedilol Versus Metoprolol in Hypertensive Patients With Type 2 Diabetes
NCT00123643	PHASE4	COMPLETED	Vascular Effects of Rosiglitazone Versus Glyburide in Type 2 Diabetic Patients
NCT00124397	PHASE4	COMPLETED	Atorvastatin and Endothelial Function in Type 2 Diabetes Mellitus (ATTEND-Study)
NCT00129233	PHASE4	COMPLETED	Comparison of Valsartan With Amlodipine in Hypertensive Patients With Glucose Intolerance
NCT00133718	PHASE4	COMPLETED	A 2 Year Trial of Patients With Type 2 Diabetes Focusing on Cardiovascular Diagnostics and Metabolic Control
NCT00135070	PHASE4	TERMINATED	Hospital In-Patient Insulin Study
NCT00141232	PHASE4	COMPLETED	Evaluating Atorvastatin With Omega-3 Fatty Acids in Cardiovascular Risk Reduction in Patients With Type 2 Diabetes
NCT00144144	PHASE4	UNKNOWN	A Study on Ca Blocker Versus AII Antagonists in Hypertension With Type 2 Diabetes
NCT00149331	PHASE4	COMPLETED	The Effects of Two Education Strategies About Insulin on Patient Preferences and Perceptions About Insulin Therapy
NCT00162357	PHASE4	COMPLETED	Post-PCI:Cardiac Imaging in Patients With Diabetes to Detect Coronary Artery Blockages Previously Opened by Angioplasty
NCT00174681	PHASE4	COMPLETED	Tulip Study: Testing the Usefulness of Lantus When Initiated Prematurely In Patients With Type 2 Diabetes
NCT00174824	PHASE4	COMPLETED	Comparison of Insulin Glargine and NPH Human Insulin in Progression of Diabetic Retinopathy in Type 2 Diabetic Patients
NCT00177398	PHASE4	COMPLETED	Effect of Glargine Insulin on Glucose Control in Hospitalized Patients Who Receive Tube Feedings
NCT00179400	PHASE4	COMPLETED	The Role of Acute Combined PPAR Alpha and Gamma Stimulation on Insulin Action in Humans
NCT00184561	PHASE4	COMPLETED	Effectiveness and Safety of Biphasic Insulin Aspart 70/30 in Subjects With Type 2 Diabetes
NCT00184626	PHASE4	COMPLETED	Comparison of Insulin Glargine Versus Biphasic Insulin Aspart 30/70 or Biphasic Insulin Aspart 30/70 in Combination With Metformin in Subjects With Type 2 Diabetes.
NCT00191178	PHASE4	COMPLETED	Effects of Insulin in Perceived Mood Symptoms in Patients With Type 2 Diabetes
NCT00191282	PHASE4	COMPLETED	Hyperglycemia and Cardiovascular Outcomes With Type 2 Diabetes
NCT00191464	PHASE4	COMPLETED	Long-Term Effects of Insulin Plus Metformin Regimens on the Overall and Postprandial Glycemic Control of Patients With Type 2 Diabetes
NCT00192803	PHASE4	UNKNOWN	Non-Insulin Dependent Diabetes Mellitus (NIDDM) and Angiotensin Converting Enzyme 2 (ACE2): Diabetic Patients Treated With Antihypertensive Drugs
NCT00202033	PHASE4	COMPLETED	Impact of Self-Monitoring Blood Glucose Frequency on Glycemic Control in Patients With Type 2 Diabetes
NCT00205660	PHASE4	COMPLETED	Changes in Adiposity, Metabolic Measures From Atypicals to Aripiprazole
NCT00212290	PHASE4	COMPLETED	Insulin Resistance and Central Nervous System (CNS) Function in Type 2 Diabetes
NCT00212303	PHASE4	COMPLETED	Exercise Training in Type 2 Diabetes and Hypertension
NCT00225342	PHASE4	WITHDRAWN	Study Protocol for Rosiglitazone Versus Gliclazide in Diabetics With Angina
NCT00238472	PHASE4	COMPLETED	A Pilot Study to Evaluate the Effects of Nateglinide vs. Glibenclamide on Renal Hemodynamics and Albumin Excretion
NCT00239538	PHASE4	COMPLETED	SMOOTH - Blood Pressure Control in Diabetic/Obese Patients
NCT00240253	PHASE4	COMPLETED	A Study Evaluating the Efficacy and Safety of Adding Symlin® to Lantus® (Insulin Glargine) in Subjects With Type 2 Diabetes