Genomes and Genes
Gene Symbol: Hnf1b
Description: HNF1 homeobox B
Alias: HNF-1-beta, HNF-1B, HNF-1Beta, Hnf1beta, LFB3, Tcf-2, Tcf2, vHNF1, hepatocyte nuclear factor 1-beta, homeoprotein LFB3, transcription factor 2
- LFB3, a heterodimer-forming homeoprotein of the LFB1 family, is expressed in specialized epitheliaV de Simone
European Molecular Biology Laboratory, Heidelberg, FRG
EMBO J 10:1435-43. 1991We have cloned and characterized a mouse cDNA coding for LFB3, a DNA binding protein containing an extra-large homeodomain...
- Hepatic nuclear factor 1 (HNF1) shows a wider distribution than products of its known target genes in developing mouseM Blumenfeld
UA 1149 du CNRS, Departement des Biotechnologies, Institut Pasteur, Paris, France
Development 113:589-99. 1991..These experiments show that, even though the HNF1 homeo-protein is essential for expression of many liver-specific genes, it cannot, by itself, force high expression levels of these genes, in non-hepatic tissues...
- HNF-1 alpha and HNF-1 beta (vHNF-1) share dimerization and homeo domains, but not activation domains, and form heterodimers in vitroD B Mendel
Beckman Center for Molecular and Genetic Medicine, Howard Hughes Medical Institute, Stanford University Medical School, California 94305
Genes Dev 5:1042-56. 1991..These studies define a pair of homeo domain proteins that have the potential to interact to produce an embryologically complex pattern of gene expression...
- Genetic analysis of late-onset type 2 diabetes in a mouse model of human complex traitH Ueda
Department of Geriatric Medicine, Osaka University Medical School, Suita, Japan
Diabetes 48:1168-74. 1999..A locus on Chr 6 was significantly linked to epididymal fat weight. A candidate disease gene (Tcf2) on Chr 11, encoding hepatic nuclear factor-1beta, was shown to have a rare sequence variant in the DNA binding ..
- HNF1alpha is involved in tissue-specific regulation of CFTR gene expressionNathalie Mouchel
Paediatric Molecular Genetics, Weatherall Institute of Molecular Medicine, Oxford University, John Radcliffe Hospital, Oxford OX3 9DS, UK
Biochem J 378:909-18. 2004..This is the first report of a transcription factor, which confers tissue specificity on the expression of this important disease-associated gene...
- The Onecut transcription factors HNF-6/OC-1 and OC-2 regulate early liver expansion by controlling hepatoblast migrationSabrina Margagliotti
Universite Catholique de Louvain, Christian de Duve Institute of Cellular Pathology, Avenue Hippocrate 75 7529, B 1200 Brussels, Belgium
Dev Biol 311:579-89. 2007..This network operating at the onset of liver development contains candidate genes for investigation of liver carcinogenesis...
- Sequence analysis of candidate genes for common susceptibility to type 1 and type 2 diabetes in miceK Yamada
Department of Geriatric Medicine, Osaka University Graduate School of Medicine, Japan
Endocr J 48:241-7. 2001..Genetic as well as functional studies have supported the possibility that Tcf2, which encodes the transcription factor, hepatocyte nuclear factor 1beta (HNF-1beta), is a candidate gene for the ..
- Hepatocyte nuclear factor 1 alpha controls renal expression of the Npt1-Npt4 anionic transporter locusClaire Cheret
Unité Expression Génétique et Maladies, Unité de Recherche Associée 1644 du Centre National de la Recherche Scientifique CNRS, Departement de Biologie du Developpement, Institut Pasteur, Paris, France
J Mol Biol 322:929-41. 2002..Transient transfection experiments confirmed that HNF1alpha directly transactivates the Npt1 promoter and that the HSS3 region contributes to this activation...
- Mutation of hepatocyte nuclear factor-1beta inhibits Pkhd1 gene expression and produces renal cysts in miceThomas Hiesberger
Division of Nephrology, Department of Internal Medicine, University of Texas Southwestern Medical Center, Dallas, Texas 75390, USA
J Clin Invest 113:814-25. 2004..These studies identify a link between two cystic disease genes, HNF1beta (MODY5) and PKHD1 (ARPKD)...
- Sox9+ ductal cells are multipotent progenitors throughout development but do not produce new endocrine cells in the normal or injured adult pancreasJanel L Kopp
Department of Pediatrics and Cellular and Molecular Medicine, University of California San Diego, La Jolla, CA 92093 0695, USA
Development 138:653-65. 2011....
- Pancreatic exocrine duct cells give rise to insulin-producing beta cells during embryogenesis but not after birthMyriam Solar
Genomic Programming of Beta Cells Laboratory, Institut d Investigacions Biomediques August Pi i Sunyer, 08036 Barcelona, Spain
Dev Cell 17:849-60. 2009..We have now used genetic labeling to fate map embryonic and adult pancreatic duct cells. We show that Hnf1beta+ cells of the trunk compartment of the early branching pancreas are precursors of acinar, duct, and endocrine ..
- vHNF1 functions in distinct regulatory circuits to control ureteric bud branching and early nephrogenesisLudmilla Lokmane
UMR7622 CNRS Université Pierre et Marie Curie, 9 quai St Bernard Bat C, 75005 Paris, France
Development 137:347-57. 2010..Here, we identify the transcription factor vHNF1 (HNF1beta) as a crucial regulator of these early developmental events...
- Hnf6 and Tcf2 (MODY5) are linked in a gene network operating in a precursor cell domain of the embryonic pancreasMiguel A Maestro
Endocrinology, Hospital Clinic, Institut d Investigacions Biomediques August Pi i Sunyer, Barcelona, Spain
Hum Mol Genet 12:3307-14. 2003..embryonic stage during which the major burst of beta-cell neogenesis takes place, pancreatic duct cells express Hnf1beta, the product of the maturity-onset diabetes of the young type 5 (MODY5) gene...
- A mitotic transcriptional switch in polycystic kidney diseaseFrancisco Verdeguer
Gene Expression, Development and Disease Laboratory, Developmental Biology Department, Institut Pasteur, Paris, France
Nat Med 16:106-10. 2010..We show here that inactivation of Hnf1b from postnatal day 10 onward does not elicit cystic dilations in tubules after their proliferative morphogenetic ..
- The diabetes gene Pdx1 regulates the transcriptional network of pancreatic endocrine progenitor cells in miceJennifer M Oliver-Krasinski
Institute for Diabetes, Obesity and Metabolism, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania, USA
J Clin Invest 119:1888-98. 2009..of the transcription factor network that regulates Ngn3 expression, SRY-box containing gene 9 (Sox9), Hnf6, Hnf1b, and forkhead box A2 (Foxa2), were decreased in homozygous mice...
- A transcriptional network in polycystic kidney diseaseLionel Gresh
Unité Expression Génétique et Maladies CNRS URA 1644, Departement de Biologie du Developpement, Institut Pasteur, Paris, France
EMBO J 23:1657-68. 2004..Hepatocyte Nuclear Factor 1beta (HNF1beta) is a homeoprotein predominantly expressed in renal, pancreatic and hepatic epithelia...
- Lack of TCF2/vHNF1 in mice leads to pancreas agenesisC Haumaitre
Biologie du Développement, Unité Mixte de Recherche 7622, Centre National de la Recherche Scientifique, Universite Pierre et Marie Curie, 9 quai St Bernard Bat C, 75005 Paris, France
Proc Natl Acad Sci U S A 102:1490-5. 2005Heterozygous mutations in the human POU-homeobox TCF2 (vHNF1, HNF1beta) gene are associated with maturity-onset diabetes of the young, type 5, and abnormal urogenital tract development...
- Integrated pathway analysis of rat urine metabolic profiles and kidney transcriptomic profiles to elucidate the systems toxicology of model nephrotoxicantsEthan Yixun Xu
Department of Safety Assessment, Merck Research Laboratories, West Point, Pennsylvania 19486, USA
Chem Res Toxicol 21:1548-61. 2008..Furthermore, this analysis suggests that renal transcription factors HNF1alpha, HNF1beta, and HIF-1 might be the central mediators of drug-induced kidney injury and adaptive response pathways.
- Mutations of HNF-1beta inhibit epithelial morphogenesis through dysregulation of SOCS-3Zhendong Ma
Departments of Internal Medicine, Molecular Biology, and Pediatrics, University of Texas Southwestern Medical Center, Dallas, TX 75390, USA
Proc Natl Acad Sci U S A 104:20386-91. 2007..Thus, HNF-1beta regulates tubulogenesis by controlling the levels of SOCS-3 expression. Manipulating the levels of SOCS-3 may be a useful therapeutic approach for human diseases induced by HNF-1beta mutations...
- Defective planar cell polarity in polycystic kidney diseaseEvelyne Fischer
Gene Expression and Disease Unit, Centre National de la Recherche Scientifique CNRS FRE 2850, Pasteur Institute, 25 rue du Docteur Roux, 75724 Paris Cedex 15, France
Nat Genet 38:21-3. 2006..These results suggest that oriented cell division dictates the maintenance of constant tubule diameter during tubular lengthening and that defects in this process trigger renal tubular enlargement and cyst formation...
- HNF1beta/TCF2 mutations impair transactivation potential through altered co-regulator recruitmentElena Barbacci
Biologie du Développement, UMR 7622, CNRS, Universite Pierre et Marie Curie, 9 Quai St Bernard, 75005 Paris, France
Hum Mol Genet 13:3139-49. 2004Mutations in the HNF1beta gene, encoding the dimeric POU-homeodomain transcription factor HNF1beta (TCF2 or vHNF1), cause various phenotypes including maturity onset diabetes of the young 5 (MODY5), and abnormalities in kidney, pancreas ..
- Mutations in hepatocyte nuclear factor-1beta and their related phenotypesE L Edghill
J Med Genet 43:84-90. 2006..Thirty HNF-1beta mutations have been reported in patients with renal cysts and other renal developmental disorders, young-onset diabetes, pancreatic atrophy, abnormal liver function tests, and genital tract abnormalities...
- Hepatocyte nuclear factor 1β induced by chemical stress accelerates cell proliferation and increases genomic instability in mouse liverKento Yoshioka
1Graduate School of Science, Toho University, Funabashi, Chiba, Japan
J Recept Signal Transduct Res 31:132-8. 2011..Our data extend the current understanding of the mechanism underlying liver regeneration against chemical stress, and identified HNF1β as a novel regulatory factor in this mechanism and as a potential initiator for carcinogenesis...
- Variant hepatocyte nuclear factor 1 is required for visceral endoderm specificationE Barbacci
U423 INSERM, Hopital Necker Enfants Malades, France U257 INSERM, ICGM, France
Development 126:4795-805. 1999..We show that variant Hepatocyte Nuclear Factor 1 (vHNF1), a homeodomain-containing transcription factor first expressed in the primitive endoderm, is required for the ..
- Essential role for the homeoprotein vHNF1/HNF1beta in visceral endoderm differentiationC Coffinier
Unite des Virus Oncogenes, CNRS URA 1644, and Unité de Biologie du Développement, CNRS URA 1960, Institut Pasteur, F 75724 Paris Cedex 15, France
Development 126:4785-94. 1999vHNF1/HNF1beta, a member of the divergent HNF1/vHNF1 homeoprotein family, is expressed in polarized epithelia of several adult organs and may participate in controlling the transcription of specific genes...
- Mutation in hepatocyte nuclear factor-1 beta gene (TCF2) associated with MODYY Horikawa
Nat Genet 17:384-5. 1997
- Expression patterns of vHNF1 and HNF1 homeoproteins in early postimplantation embryos suggest distinct and sequential developmental rolesS Cereghini
Unité de Génétique de la Différenciation, UA 1149 du CNRS, Institut Pasteur, Paris, France
Development 116:783-97. 1992The homeoproteins HNF1 (LFB1/HNF1-A) and vHNF1 (LFB3/HNF1 beta) interact with an essential control element of a group of liver-specific genes...
- Hepatocyte nuclear factor 1 inactivation results in hepatic dysfunction, phenylketonuria, and renal Fanconi syndromeM Pontoglio
Unite des Virus Oncogenes, Departement des Biotechnologies, InstitutPasteur, Paris, France
Cell 84:575-85. 1996..The resulting massive urinary glucose loss leads to energy and water wasting. HNF1-deficient mice may provide a model for human renal Fanconi syndrome...
- Role of the hepatocyte nuclear factor-1beta (HNF-1beta) C-terminal domain in Pkhd1 (ARPKD) gene transcription and renal cystogenesisThomas Hiesberger
Department of Internal Medicine, University of Texas Southwestern Medical Center, 5323 Harry Hines Blvd, Dallas, Texas 75390 8856, USA
J Biol Chem 280:10578-86. 2005..Expression of HNF-1alpha in proximal tubules may protect against cystogenesis...
- The mammal-specific Pdx1 Area II enhancer has multiple essential functions in early endocrine-cell specification and postnatal β-cell maturationYu Ping Yang
Vanderbilt University Program in Developmental Biology and Department of Cell and Developmental Biology, Vanderbilt University, Nashville, TN 37232, USA
Development . 2016..Therefore, trans-regulatory integration through the mammal-restricted Area II mediates a surprisingly extensive range of progenitor and β-cell-specific functions of the Pdx1 gene...
- Transcription factors SOX4 and SOX9 cooperatively control development of bile ductsAlexis Poncy
Universite Catholique de Louvain, de Duve Institute, Avenue Hippocrate 75 B1 7503, Brussels 1200, Belgium Electronic address
Dev Biol 404:136-48. 2015..We conclude that SOX4 is a new regulator of liver development and that it exerts a pleiotropic control on bile duct development in cooperation with SOX9. ..
- The role of tumor necrosis factor alpha in regulating the expression of Tamm-Horsfall Protein (uromodulin) in thick ascending limbs during kidney injuryMonique Heitmeier
Washington University in St Louis, St Louis, MO, USA
Am J Nephrol 40:458-67. 2014..Tumor necrosis factor alpha (TNFα) is a cytokine endogenously expressed by the TAL and is also induced by AKI. Therefore, we hypothesized that TNFα is a key regulator of THP expression...
- Hnf-1β transcription factor is an early hif-1α-independent marker of epithelial hypoxia and controls renal repairStanislas Faguer
Département de Néphrologie et Transplantation d organes, CHU Rangueil, Toulouse, France
PLoS ONE 8:e63585. 2013..In addition, early decrease in Hnf1b expression is associated with the transient over-expression of one of its main target genes, the suppressor of ..
- Transcriptional regulation of human and mouse organic anion transporter 1 by hepatocyte nuclear factor 1 alpha/betaTakami Saji
Department of Molecular Pharmacokinetics, Graduate School of Pharmaceutical Sciences, The University of Tokyo, 7 3 1 Hongo, Bunkyo ku, Tokyo, USA
J Pharmacol Exp Ther 324:784-90. 2008..2006). Forced expression of HNF1alpha alone or both HNF1alpha and HNF1beta enhanced the activity of human and mouse OAT1 promoters in the transactivation assays, whereas HNF1beta alone was ..
- Neurogenin3 cooperates with Foxa2 to autoactivate its own expressionMiriam Ejarque
Diabetes and Obesity Laboratory, Institut d Investigacions Biomèdiques August Pi i Sunyer Hospital Clínic, 08036 Barcelona, Spain
J Biol Chem 288:11705-17. 2013....
- Wnt5a signaling mediates biliary differentiation of fetal hepatic stem/progenitor cells in miceKei Kiyohashi
Department of Gastroenterology and Hepatology, Tokyo, Japan
Hepatology 57:2502-13. 2013....
- Spatiotemporal patterns of multipotentiality in Ptf1a-expressing cells during pancreas organogenesis and injury-induced facultative restorationFong Cheng Pan
Vanderbilt University Program in Developmental Biology, Department of Cell and Developmental Biology, Vanderbilt University Medical Center, Nashville, TN 37232, USA
Development 140:751-64. 2013..Thus, we show that acinar cells, without exogenously introduced factors, can regain aspects of embryonic multipotentiality under injury, and convert into mature β-cells...
- Pancreas-enriched miRNA refines endocrine cell differentiationSharon Kredo-Russo
Department of Molecular Genetics, Weizmann Institute of Science, Rehovot 76100, Israel
Development 139:3021-31. 2012..Furthermore, Pax6 downregulation reversed the effect of miR-7 knockdown on insulin promoter activity. These data suggest a novel miR-7-based circuit that ensures precise control of endocrine cell differentiation...
- A Notch-dependent molecular circuitry initiates pancreatic endocrine and ductal cell differentiationHung Ping Shih
Department of Pediatrics and Cellular and Molecular Medicine, University of California San Diego, La Jolla, CA 92093 0695, USA
Development 139:2488-99. 2012....
- Cardiovascular defects in a mouse model of HOXA1 syndromeNadja Makki
Howard Hughes Medical Institute and Department of Human Genetics, University of Utah, Salt Lake City, UT 84112 5331, USA
Hum Mol Genet 21:26-31. 2012..Thus, our data allow us to suggest a model in which Hoxa1 regulates heart development through its influence on cardiac NCCs, providing insight into the mechanisms underlying the human disease...
- Ngn3(+) endocrine progenitor cells control the fate and morphogenesis of pancreatic ductal epitheliumJudith Magenheim
Department of Developmental Biology and Cancer Research, The Institute for Medical Research Israel Canada, The Hebrew University Hadassah Medical School, Jerusalem 91120, Israel
Dev Biol 359:26-36. 2011..These results uncover a novel layer of regulation coordinating pancreas morphogenesis and endocrine/exocrine differentiation, and suggest ways to enhance the yield of beta cells from stem cells...
- HNF-1 alpha and HNF-1 beta expression in mouse intestinal cryptsM S Serfas
Department of Genetics, University of Illinois College of Medicine, Chicago 60612
Am J Physiol 265:G506-13. 1993..Although HNF-1 is necessary for transcription of the AFP gene, other factors must be involved in eliciting the cell type-specific pattern of AFP expression in the intestine...
- Abnormal splicing of hepatocyte nuclear factor-1 beta in the renal cysts and diabetes syndromeL W Harries
Molecular Genetics, Institute of Biomedical and Clinical Science, Peninsula Medical School, Exeter, UK
Diabetologia 47:937-42. 2004..This is the aim of the present study...
- Zyxin regulates migration of renal epithelial cells through activation of hepatocyte nuclear factor-1βYun Hee Choi
Department of Internal Medicine, University of Texas Southwestern Medical Center, Dallas, TX 75390, USA
Am J Physiol Renal Physiol 305:F100-10. 2013..These findings reveal a novel pathway by which extracellular signals are transmitted to the nucleus to regulate the activity of a transcription factor that is essential for renal epithelial differentiation. ..
- De novo lumen formation and elongation in the developing nephron: a central role for afadin in apical polarityZhufeng Yang
Department of Medicine, University of Texas Southwestern Medical Center, Dallas, TX 75390, USA
Development 140:1774-84. 2013....
- HNF1B controls proximal-intermediate nephron segment identity in vertebrates by regulating Notch signalling components and Irx1/2Claire Heliot
Inserm Unité 969, 9 quai St Bernard Bat C, 75005 Paris, France
Development 140:873-85. 2013..The regulatory circuits involved in initial nephron patterning are poorly understood. We report here that HNF1B, a transcription factor known to be involved in ureteric bud branching and initiation of nephrogenesis, has an ..
- Hepatocyte nuclear factor 1β controls nephron tubular developmentFilippo Massa
Expression Génique, Développement et Maladies EGDM Team, Inserm U1016, CNRS UMR 8104, Université Paris Descartes Institut Cochin Département deGénétique et Développement, 75014 Paris, France
Development 140:886-96. 2013..Mutations in HNF1B (OMIM #137920) are frequently found in patients with developmental renal pathologies, the mechanisms of which have ..
- Notch-mediated patterning and cell fate allocation of pancreatic progenitor cellsSolomon Afelik
Lerner Research Institute, Cleveland Clinic, Cleveland, OH 44195, USA
Development 139:1744-53. 2012..Mechanistically, these observations could be explained by a requirement of Notch for the expression of the trunk determination gene Nkx6.1. This was supported by the finding of direct binding of RBP-jκ to the Nkx6.1 proximal promoter...
- Hepatocyte nuclear factor 4 alpha is related to survival of the condensed mesenchyme in the developing mouse kidneyTomonori Kanazawa
Laboratory of Anatomy, Graduate School of Veterinary Medicine, Hokkaido University, Sapporo 060 0818, Japan
Dev Dyn 239:1145-54. 2010..These results suggest the possibility that Hnf4alpha plays an important role in the regulation of the cell survival at the CM stage in nephrogenesis...
- The onecut transcription factor HNF6 is required for normal development of the biliary tractFrederic Clotman
Hormone and Metabolic Research Unit, Institute of Cellular Pathology and Université catholique de Louvain, Avenue Hippocrate 75, B 1200 Brussels, Belgium
Development 129:1819-28. 2002..The morphology of the intrahepatic bile ducts was identical to that seen in mice whose Hnf1beta gene has been conditionally inactivated in the liver...
- Paracrine action of FGF4 during periimplantation development maintains trophectoderm and primitive endodermSarah N Goldin
Genesis 36:40-7. 2003..Thus, postimplantation lethality of Fgf4(-/-) embryos likely results from the failure of proper differentiation and function of extraembryonic cell types...
- Species-specific differences in the expression of the HNF1A, HNF1B and HNF4A genesLorna W Harries
Institute of Biomedical and Clinical Sciences, Peninsula Medical School, University of Exeter, Exeter, United Kingdom
PLoS ONE 4:e7855. 2009The HNF1A, HNF1B and HNF4A genes are part of an autoregulatory network in mammalian pancreas, liver, kidney and gut...
- Dynamic signaling network for the specification of embryonic pancreas and liver progenitorsEwa Wandzioch
Cell and Developmental Biology Program, Fox Chase Cancer Center, 333 Cottman Avenue, Philadelphia, PA 19111, USA
Science 324:1707-10. 2009..These findings may enhance progenitor cell specification from stem cells for biomedical purposes and can help explain incomplete programming in stem cell differentiation protocols...
- Notch2 signaling promotes biliary epithelial cell fate specification and tubulogenesis during bile duct development in miceJan S Tchorz
Department of Biomedicine, Institute of Physiology, University of Basel, Switzerland
Hepatology 50:871-9. 2009..Ectopic ducts in lobular regions did not persist into adulthood, indicating that local signals in the portal environment are important for maintaining bile ducts...
- Quantitative gene expression profiling reveals a fetal hepatic phenotype of murine ES-derived hepatocytesAndrea Jochheim
Hannover Medical School, Center of Internal Medicine, Department of Gastroenterology, Hepatology and Endocrinology, Hannover, Germany
Int J Dev Biol 48:23-9. 2004..With sophisticated differentiation protocols, hepatic gene expression can be induced in a proportion of ES cells with gene expression patterns similar to early fetal liver...
- Intrahepatic bile ducts develop according to a new mode of tubulogenesis regulated by the transcription factor SOX9Aline Antoniou
Universite Catholique de Louvain, de Duve Institute, Brussels, Belgium
Gastroenterology 136:2325-33. 2009..In the embryo, hepatoblasts differentiate to cholangiocytes, which give rise to the bile ducts. Here, we investigated duct development in mouse liver and characterized the role of the SRY-related HMG box transcription factor 9 (SOX9)...
- Hepatocyte nuclear factor 1alpha and beta control terminal differentiation and cell fate commitment in the gut epitheliumAnna D'Angelo
Expression Génique, Développement et Maladies Equipe 26 INSERM U567 CNRS UMR 8104 Université Paris Descartes Institut Cochin Dpt Génétique et Développement, 75014 Paris, France
Development 137:1573-82. 2010..With the use of a conditional inactivation system, we generated mice in which Hnf1b is specifically inactivated in the intestinal epithelium on a wild-type or Hnf1a(-/-) genetic background...
- The mouse alpha-albumin (afamin) promoter is differentially regulated by hepatocyte nuclear factor 1α and hepatocyte nuclear factor 1βHua Liu
Department of Microbiology, Immunology, and Molecular Genetics, University of Kentucky, Lexington, Kentucky 40536, USA
DNA Cell Biol 30:137-47. 2011....
- The mouse KRAB zinc-finger protein CHATO is required in embryonic-derived tissues to control yolk sac and placenta morphogenesisMaho Shibata
Department of Molecular Biology and Genetics, Cornell University, 259 Biotechnology Building, Ithaca, NY 14853, USA
Dev Biol 349:331-41. 2011..Our results support previously undescribed roles of the extraembryonic mesoderm in yolk sac morphogenesis and in the closure of the ectoplacental cavity and identify a novel role of ZFP568 in the development of extraembryonic tissues...
- Cystic renal neoplasia following conditional inactivation of apc in mouse renal tubular epitheliumChao Nan Qian
Laboratories of Cancer Genetics and Cell Signaling and Carcinogenesis, Van Andel Research Institute, Grand Rapids, Michigan 49508, USA
J Biol Chem 280:3938-45. 2005..Our results confirm an important role for proper regulation of Wnt/beta-catenin signaling in renal development and provide evidence that dysregulation of the pathway can initiate tumorigenesis in the kidney...
- Sox9-haploinsufficiency causes glucose intolerance in miceClaire L Dubois
Department of Pediatrics and Cellular and Molecular Medicine, University of California San Diego, La Jolla, California, United States of America
PLoS ONE 6:e23131. 2011..it has been suggested that Sox9 controls expression of a network of important developmental regulators, including Tcf2/MODY5, Hnf6, and Foxa2, in pancreatic progenitor cells...
- Hepatocyte nuclear factor 3beta (Foxa2) is dispensable for maintaining the differentiated state of the adult hepatocyteN J Sund
Department of Genetics, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104 6145, USA
Mol Cell Biol 20:5175-83. 2000..This is the first example of such functional dichotomy for a tissue specification transcription factor...
- ME1 and GE1: basic helix-loop-helix transcription factors expressed at high levels in the developing nervous system and in morphogenetically active regionsT Neuman
Department of Anatomy and Neurobiology, Colorado State University, Fort Collins 80523
Eur J Neurosci 5:311-8. 1993....
- Localization of sequences related to the human RAD6 DNA repair gene on mouse chromosomes 11 and 13M L Roller
Department of Human Genetics, University of Michigan Medical School, Ann Arbor 48109 0618, USA
Mamm Genome 6:305-6. 1995
- vHNF1 is expressed in epithelial cells of distinct embryonic origin during development and precedes HNF1 expressionM O Ott
Departement de Biologie Moleculaire, Institut Pasteur, Paris, France
Mech Dev 36:47-58. 1991HNF1 (Hepatic Nuclear Factor 1) and vHNF1 are transcriptional regulators containing a highly divergent homeodomain...
- Sall4 regulates cell fate decision in fetal hepatic stem/progenitor cellsTsunekazu Oikawa
Division of Stem Cell Therapy, Center for Stem Cell and Regenerative Medicine, The Institute of Medical Science, University of Tokyo, Tokyo, Japan
Gastroenterology 136:1000-11. 2009..The expression and functional roles of Sall4 during liver development have not been elucidated. We here provide their first description in hepatoblasts...
- Shifting boundaries of retinoic acid activity control hindbrain segmental gene expressionIoan Ovidiu Sirbu
OncoDevelopmental Biology Program, Burnham Institute, 10901 North Torrey Pines Road, La Jolla, CA 92037, USA
Development 132:2611-22. 2005..widespread induction up to r4 and for r3/r5 repression, but RA has previously been detected only from r5-r8, and vHnf1 is required for repression of Hoxb1 posterior to r4 in zebrafish...
- The vHNF1 homeodomain protein establishes early rhombomere identity by direct regulation of Kreisler expressionFlorence A Kim
Samuel Lunenfeld Research Institute, Mt Sinai Hospital, 600 University Avenue, Toronto, Ont, Canada
Mech Dev 122:1300-9. 2005..variant Hepatocyte Nuclear Factor 1 (vHNF1/HNF1beta/LF-3B) is transiently expressed in the r5/r6 domain of 0-10 somite stage embryos and a vHNF1binding site within ..
- Cardiomyocyte-specific Bcl-2 overexpression attenuates ischemia-reperfusion injury, immune response during acute rejection, and graft coronary artery diseaseMasashi Tanaka
Department of Cardiothoracic Surgery, Falk Cardiovascular Research Center, 300 Pasteur Dr CVRB, Stanford University School of Medicine, Stanford, CA 94305 5407, USA
Blood 104:3789-96. 2004..In conclusion, our findings suggest that the modulation of Bcl-2 expression by pharmacologic up-regulation or gene transfer may be of clinical benefit in the short- and long-term function of cardiac allografts...
- Visceral endoderm function is regulated by quaking and required for vascular developmentBrenda L Bohnsack
Department of Molecular and Cellular Biology, Baylor College of Medicine, Houston, Texas 77030, USA
Genesis 44:93-104. 2006..Although these defects were rescued by exogenous RA, visceral endoderm function or vascular remodeling were not restored. Thus, we conclude that qkI regulates visceral endoderm function, which is critical for vascular remodeling...
- The transcription factor hepatocyte nuclear factor-6 controls the development of pancreatic ducts in the mouseChristophe E Pierreux
Hormone and Metabolic Research Unit, Université catholique de Louvain and Institute of Cellular Pathology, Brussels, Belgium
Gastroenterology 130:532-41. 2006..Here, we investigate the transcriptional mechanisms that control pancreatic duct development by addressing the role of the transcription factor hepatocyte nuclear factor (HNF)-6...
- Role of TIF1alpha as a modulator of embryonic transcription in the mouse zygoteMaria Elena Torres-Padilla
The Wellcome Trust Cancer Research UK Gurdon Institute, University of Cambridge, Cambridge CB2 1QR, England, UK
J Cell Biol 174:329-38. 2006..These studies indicate that TIF1alpha is a factor that modulates the expression of a set of genes during the first wave of genome activation in the mouse embryo...
- Plasticity and expanding complexity of the hepatic transcription factor network during liver developmentIrene Kyrmizi
Institute of Molecular Biology and Biotechnology, Foundation for Research and Technology, Hellas, 711 10 Herakleion, Crete, Greece
Genes Dev 20:2293-305. 2006..The results illustrate the remarkable flexibility of a self-sustaining transcription factor network, built up by complex dominant and redundant regulatory motifs in developing hepatocytes...
- Snail activation disrupts tissue homeostasis and induces fibrosis in the adult kidneyAgnes Boutet
Instituto de Neurociencias de Alicante, CSIC UMH, Sant Joan d Alacant, Alicante, Spain
EMBO J 25:5603-13. 2006..Significantly, Snail is also activated in patients with renal fibrosis. Thus, Snail expression is suppressed during renal development and it must remain silent in the mature kidney where its aberrant activation leads to fibrosis...
- Snail2, a mediator of epithelial-mesenchymal transitions, expressed in progenitor cells of the developing endocrine pancreasJ Michael Rukstalis
Laboratory of Molecular Endocrinology, Massachusetts General Hospital, Harvard Medical School and Howard Hughes Medical Institute, Boston, MA 02114, USA
Gene Expr Patterns 7:471-9. 2007..EMT-like events appear to be involved in the development of the mammalian pancreas in vivo...
- Glomerulocystic kidney disease in mice with a targeted inactivation of Wwtr1Zakir Hossain
Epithelial Cell Biology Laboratory, Institute of Molecular and Cell Biology, 61 Biopolis Drive, Republic of Singapore 138673
Proc Natl Acad Sci U S A 104:1631-6. 2007..Thus, Wwtr1 is critical for the integrity of renal cilia and its absence in mice leads to the development of renal cysts, indicating that Wwtr1 may represent a candidate gene for polycystic kidney disease in humans...
- The role for HNF-1beta-targeted collectrin in maintenance of primary cilia and cell polarity in collecting duct cellsYanling Zhang
Department of Medicine and Clinical Science, Okayama University Graduate School of Medicine, Okayama, Japan
PLoS ONE 2:e414. 2007..The transcriptional hierarchy between HNF-1beta and PKD (polycystic kidney disease) genes expressed in the primary cilia of collecting duct cells has been suggested, and collectrin is one of such HNF-1beta regulated genes...
- The homeobox gene Hhex is essential for proper hepatoblast differentiation and bile duct morphogenesisMichael P Hunter
Department of Pediatrics, Yale University School of Medicine, 333 Cedar Street, New Haven, CT 06510, USA
Dev Biol 308:355-67. 2007..embryonic liver (Alfp-Cre;Hhex(d2,3/-)) caused irregular development of intrahepatic bile ducts and an absence of Hnf1beta in many (cystic) biliary epithelial cells, which resulted in a slow, progressive form of polycystic liver disease ..
- A genetic screen in zebrafish identifies cilia genes as a principal cause of cystic kidneyZhaoxia Sun
MIT, Center for Cancer Research and Biology Department, Building E17 Room 340, 77 Massachusetts Avenue, Cambridge, MA 02139, USA
Development 131:4085-93. 2004..Two of these genes, vhnf1 (tcf2) and pkd2, are already associated with human cystic kidney diseases...
- Expression of HNF-4alpha (MODY1), HNF-1beta (MODY5), and HNF-1alpha (MODY3) proteins in the developing mouse pancreasTakao Nammo
Department of Metabolic Medicine, Graduate School of Medicine, B5, Osaka University, 2 2 Yamada oka, Suita, Osaka 565 0871, Japan
Gene Expr Patterns 8:96-106. 2008..Hara, M., Furuta, H., Hinokio, Y., Cockburn, B.N. et al., 1997. Mutation in hepatocyte nuclear factor-1beta gene (TCF2) associated with MODY. Nat. Genet. 17, 384-385]...
- Morphogenetic competence of HNF4 alpha-deficient mouse hepatic cellsGraham P Hayhurst
Unité de Génétique de la Différenciation, Centre National de la Recherche Scientifique, Institut Pasteur, Paris, France
J Hepatol 49:384-95. 2008..To specify roles of HNF 4 alpha in mouse liver development, we have analyzed the ex vivo morphogenetic potential of HNF4 alpha-null embryonic hepatic cells...
- Mapping and promoter sequencing of HNF-1beta gene in diabetes-prone and -resistant miceH Ueda
Department of Geriatric Medicine, Osaka University Medical School, 2 2 Yamadaoka, Suita, 565 0871, Osaka, Japan
Diabetes Res Clin Pract 53:67-71. 2001By using a novel single nucleotide polymorphism (SNP) in the coding sequence, the chromosomal location of Tcf2, encoding hepatic nuclear factor (HNF)-1beta, was determined in F2 intercrosses between Nagoya-Shibata-Yasuda (NSY) mice, an ..
- Direct regulation of vHnf1 by retinoic acid signaling and MAF-related factors in the neural tubeMarie Pouilhe
INSERM, U784, 46 rue d Ulm, 75230 Paris Cedex 05, France
Dev Biol 309:344-57. 2007The homeodomain transcription factor vHNF1 plays an essential role in the patterning of the caudal segmented hindbrain, where it participates in the definition of the boundary between rhombomeres (r) 4 and 5 and in the specification of ..
- The anterior visceral endoderm of the mouse embryo is established from both preimplantation precursor cells and by de novo gene expression after implantationMaria Elena Torres-Padilla
The Wellcome Trust Cancer Research UK Gurdon Institute, University of Cambridge, Tennis Court Road, Cambridge CB2 1QN, UK
Dev Biol 309:97-112. 2007..Together, these results suggest that the orientation of the anterior-posterior axis is already anticipated before AVE migration...
- Defects in brain patterning and head morphogenesis in the mouse mutant Fused toesIsabelle Anselme
Biologie du Développement, CNRS UMR7622, Universite Pierre et Marie Curie, 9 quai Saint Bernard, 75252 Paris Cedex 05, France
Dev Biol 304:208-20. 2007..Given the diversity, localisation and nature of the defects, we propose that some of them are caused by the elimination of the IrxB cluster, while others result from the loss of one or several of the Fts, Ftm and Fto genes...
- Newly identified paralogous groups on mouse chromosomes 5 and 11 reveal the age of a T-box cluster duplicationI Ruvinsky
Lewis Thomas Laboratory, Department of Molecular Biology, Princeton University, New Jersey 08544 1014, USA
Genomics 40:262-6. 1997..This work facilitates our understanding of the status of the T-box gene family in different vertebrate lineages and also defines a novel paralogy group within the mouse genome...
- Complex genetic control of HDL levels in mice in response to an atherogenic diet. Coordinate regulation of HDL levels and bile acid metabolismD Machleder
Department of Medicine, University of California, Los Angeles 90095, USA
J Clin Invest 99:1406-19. 1997..Furthermore, we observed that two of the loci for C7AH expression contributed to differences in gallstone formation between these strains...
- Notch signaling controls liver development by regulating biliary differentiationYiwei Zong
Abramson Family Cancer Research Institute, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA
Development 136:1727-39. 2009..These results reconcile previous conflicting reports about the role of Notch during liver development and suggest that Notch acts by coordinating biliary differentiation and morphogenesis...
- HNF-1beta regulates transcription of the PKD modifier gene Kif12Yimei Gong
Department of Internal Medicine, University of Texas Southwestern Medical Center, Dallas, TX 75390, USA
J Am Soc Nephrol 20:41-7. 2009..Because kinesin-12 family members participate in orienting cell division, downregulation of Kif12 may underlie the abnormal planar cell polarity observed in cystic kidney diseases...
- Signaling hierarchy downstream of retinoic acid that independently regulates vascular remodeling and endothelial cell proliferationBrenda L Bohnsack
Department of Molecular and Cellular Biology, Children s Nutrition Research Center, Center for Cell and Gene Therapy, Baylor College of Medicine, Houston, Texas 77030, USA
Genes Dev 18:1345-58. 2004....
- Comprehensive search for HNF-1beta-regulated genes in mouse hepatoma cells perturbed by transcription regulatory factor-targeted RNAiTaku Tanaka
Division of Genomics, Science of Biological Supramolecular Systems, Graduate School of Integrated Science, Yokohama City University, 1 7 29 Suehiro cho, Tusurumi Ku, Yokohama, Kanagawa 230 0045, Japan
Nucleic Acids Res 32:2740-50. 2004....
- The gene coding for variant hepatic nuclear factor 1 (Tcf-2), maps between the Edp-1 and Erba genes on mouse chromosome 11I J Karolyi
Department of Human Genetics, University of Michigan Medical School, Ann Arbor 48109 0618
Mamm Genome 3:184-5. 1992
- Expression of Groucho/TLE proteins during pancreas developmentBrad G Hoffman
Department of Cancer Endocrinology, BC Cancer Research Center, 675 West 10th Avenue, Vancouver, BC, V5Z 1L3, Canada
BMC Dev Biol 8:81. 2008..Moreover, little is known about the ability of Gro/TLE proteins to interact with transcription factors in the pancreas...
- A vHNF1/TCF2-HNF6 cascade regulates the transcription factor network that controls generation of pancreatic precursor cellsAurélie V Poll
Hormone and Metabolic Research Unit, Université catholique de Louvain and Institute of Cellular Pathology, Avenue Hippocrate 75 7529, B 1200 Brussels, Belgium
Diabetes 55:61-9. 2006..Among the latter, variant HNF1 (vHNF1)/TCF2, which is expressed before HNF6 in the endoderm, was found to be critical for HNF6 expression...
- Regulation of kidney-specific Ksp-cadherin gene promoter by hepatocyte nuclear factor-1betaYun Bai
Division of Nephrology, Department of Internal Medicine, University of Texas Southwestern Medical Center at Dallas, 75390, USA
Am J Physiol Renal Physiol 283:F839-51. 2002..Mutations of HNF-1beta, as occur in humans with inherited renal cysts and diabetes, may cause dysregulated Ksp-cadherin promoter activity...
- Linkage mapping of murine homolog of the yeast SPT6 gene to MMU11B1P W Chiang
Department of Pediatrics, University of Michigan Medical Center, Ann Arbor 48109 0650, USA
Mamm Genome 7:459-60. 1996
- Regulation of tissue-specific expression of the human and mouse urate transporter 1 gene by hepatocyte nuclear factor 1 alpha/beta and DNA methylationRyota Kikuchi
Department of Molecular Pharmacokinetics, Graduate School of Pharmaceutical Sciences, The University of Tokyo, 7 3 1 Hongo, Bunkyo ku, Tokyo 113 0033, Japan
Mol Pharmacol 72:1619-25. 2007..Hepatocyte nuclear factor 1 alpha (HNF1alpha) and HNF1beta positively regulate minimal promoter activity of the URAT1 gene as shown by reporter gene assays...