Hnf1b

Summary

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
Species: mouse

Top Publications

  1. pmc LFB3, a heterodimer-forming homeoprotein of the LFB1 family, is expressed in specialized epithelia
    V de Simone
    European Molecular Biology Laboratory, Heidelberg, FRG
    EMBO J 10:1435-43. 1991
  2. ncbi Hepatic nuclear factor 1 (HNF1) shows a wider distribution than products of its known target genes in developing mouse
    M Blumenfeld
    UA 1149 du CNRS, Departement des Biotechnologies, Institut Pasteur, Paris, France
    Development 113:589-99. 1991
  3. ncbi HNF-1 alpha and HNF-1 beta (vHNF-1) share dimerization and homeo domains, but not activation domains, and form heterodimers in vitro
    D 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
  4. ncbi Genetic analysis of late-onset type 2 diabetes in a mouse model of human complex trait
    H Ueda
    Department of Geriatric Medicine, Osaka University Medical School, Suita, Japan
    Diabetes 48:1168-74. 1999
  5. pmc HNF1alpha is involved in tissue-specific regulation of CFTR gene expression
    Nathalie Mouchel
    Paediatric Molecular Genetics, Weatherall Institute of Molecular Medicine, Oxford University, John Radcliffe Hospital, Oxford OX3 9DS, UK
    Biochem J 378:909-18. 2004
  6. ncbi The Onecut transcription factors HNF-6/OC-1 and OC-2 regulate early liver expansion by controlling hepatoblast migration
    Sabrina 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
  7. ncbi Sequence analysis of candidate genes for common susceptibility to type 1 and type 2 diabetes in mice
    K Yamada
    Department of Geriatric Medicine, Osaka University Graduate School of Medicine, Japan
    Endocr J 48:241-7. 2001
  8. ncbi Hepatocyte nuclear factor 1 alpha controls renal expression of the Npt1-Npt4 anionic transporter locus
    Claire 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
  9. pmc Mutation of hepatocyte nuclear factor-1beta inhibits Pkhd1 gene expression and produces renal cysts in mice
    Thomas 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
  10. pmc Sox9+ ductal cells are multipotent progenitors throughout development but do not produce new endocrine cells in the normal or injured adult pancreas
    Janel 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

Scientific Experts

  • Qiuwei Xu
  • Alexis Poncy
  • Vishal Patel
  • Lorna W Harries
  • Thomas Hiesberger
  • M Yaniv
  • Solomon Afelik
  • Masashi Tanaka
  • S Cereghini
  • Marco Pontoglio
  • Peter Igarashi
  • Evelyne Fischer
  • Christophe E Pierreux
  • Frederic P Lemaigre
  • Hung Ping Shih
  • Claire L Dubois
  • Serge Garbay
  • Frederic Clotman
  • Guy G Rousseau
  • Ludmilla Lokmane
  • Antonia Doyen
  • Maria Elena Torres-Padilla
  • Claire Heliot
  • Philip A Seymour
  • Maike Sander
  • Janel L Kopp
  • Patrick Jacquemin
  • Lionel Gresh
  • Brenda L Bohnsack
  • Ben Z Stanger
  • Yiwei Zong
  • Aline Antoniou
  • Yimei Gong
  • Zhendong Ma
  • Ryota Kikuchi
  • Yu Ping Yang
  • Frank J Gonzalez
  • H Ueda
  • Christopher V E Wright
  • Mark A Magnuson
  • Monique Heitmeier
  • Aurélie V Poll
  • Jorge Ferrer
  • Stanislas Faguer
  • Filippo Massa
  • Kei Kiyohashi
  • Zhufeng Yang
  • Fong Cheng Pan
  • Miriam Ejarque
  • Yun Hee Choi
  • Sharon Kredo-Russo
  • Nadja Makki
  • Maho Shibata
  • Xiaobo Xu
  • Kento Yoshioka
  • Miguel A Maestro
  • Hiromitsu Nakauchi
  • Akihide Kamiya
  • Hua Liu
  • Judith Magenheim
  • Sei Kakinuma
  • Harry Heimberg
  • Tomonori Kanazawa
  • Anna D'Angelo
  • Francisco Verdeguer
  • Ewa Wandzioch
  • Myriam Solar
  • Jennifer M Oliver-Krasinski
  • Peggy Raynaud
  • Tsunekazu Oikawa
  • Jan S Tchorz
  • Brad G Hoffman
  • Takami Saji
  • Ethan Yixun Xu
  • Klaus H Kaestner
  • Graham P Hayhurst
  • Takao Nammo
  • Francois Tronche
  • Kenji Fukui
  • Sabine Cordi
  • Yuichi Sugiyama
  • Kazuya Yamagata
  • Insook Kim
  • Hiroyuki Kusuhara
  • Isabelle Anselme
  • Michael P Hunter
  • Marie Pouilhe
  • Yanling Zhang
  • Zakir Hossain
  • J Michael Rukstalis

Detail Information

Publications98

  1. pmc LFB3, a heterodimer-forming homeoprotein of the LFB1 family, is expressed in specialized epithelia
    V de Simone
    European Molecular Biology Laboratory, Heidelberg, FRG
    EMBO J 10:1435-43. 1991
    We have cloned and characterized a mouse cDNA coding for LFB3, a DNA binding protein containing an extra-large homeodomain...
  2. ncbi Hepatic nuclear factor 1 (HNF1) shows a wider distribution than products of its known target genes in developing mouse
    M 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...
  3. ncbi HNF-1 alpha and HNF-1 beta (vHNF-1) share dimerization and homeo domains, but not activation domains, and form heterodimers in vitro
    D 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...
  4. ncbi Genetic analysis of late-onset type 2 diabetes in a mouse model of human complex trait
    H 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 ..
  5. pmc HNF1alpha is involved in tissue-specific regulation of CFTR gene expression
    Nathalie 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...
  6. ncbi The Onecut transcription factors HNF-6/OC-1 and OC-2 regulate early liver expansion by controlling hepatoblast migration
    Sabrina 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...
  7. ncbi Sequence analysis of candidate genes for common susceptibility to type 1 and type 2 diabetes in mice
    K 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 ..
  8. ncbi Hepatocyte nuclear factor 1 alpha controls renal expression of the Npt1-Npt4 anionic transporter locus
    Claire 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...
  9. pmc Mutation of hepatocyte nuclear factor-1beta inhibits Pkhd1 gene expression and produces renal cysts in mice
    Thomas 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)...
  10. pmc Sox9+ ductal cells are multipotent progenitors throughout development but do not produce new endocrine cells in the normal or injured adult pancreas
    Janel 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
    ....
  11. doi Pancreatic exocrine duct cells give rise to insulin-producing beta cells during embryogenesis but not after birth
    Myriam 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 ..
  12. doi vHNF1 functions in distinct regulatory circuits to control ureteric bud branching and early nephrogenesis
    Ludmilla 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...
  13. ncbi Hnf6 and Tcf2 (MODY5) are linked in a gene network operating in a precursor cell domain of the embryonic pancreas
    Miguel 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...
  14. pmc A mitotic transcriptional switch in polycystic kidney disease
    Francisco 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 ..
  15. pmc The diabetes gene Pdx1 regulates the transcriptional network of pancreatic endocrine progenitor cells in mice
    Jennifer 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...
  16. pmc A transcriptional network in polycystic kidney disease
    Lionel 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...
  17. pmc Lack of TCF2/vHNF1 in mice leads to pancreas agenesis
    C 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. 2005
    Heterozygous 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...
  18. doi Integrated pathway analysis of rat urine metabolic profiles and kidney transcriptomic profiles to elucidate the systems toxicology of model nephrotoxicants
    Ethan 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.
  19. pmc Mutations of HNF-1beta inhibit epithelial morphogenesis through dysregulation of SOCS-3
    Zhendong 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...
  20. ncbi Defective planar cell polarity in polycystic kidney disease
    Evelyne 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...
  21. ncbi HNF1beta/TCF2 mutations impair transactivation potential through altered co-regulator recruitment
    Elena 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. 2004
    Mutations 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 ..
  22. pmc Mutations in hepatocyte nuclear factor-1beta and their related phenotypes
    E 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...
  23. doi Hepatocyte nuclear factor 1β induced by chemical stress accelerates cell proliferation and increases genomic instability in mouse liver
    Kento 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...
  24. ncbi Variant hepatocyte nuclear factor 1 is required for visceral endoderm specification
    E 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 ..
  25. ncbi Essential role for the homeoprotein vHNF1/HNF1beta in visceral endoderm differentiation
    C 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. 1999
    vHNF1/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...
  26. ncbi Mutation in hepatocyte nuclear factor-1 beta gene (TCF2) associated with MODY
    Y Horikawa
    Nat Genet 17:384-5. 1997
  27. ncbi Expression patterns of vHNF1 and HNF1 homeoproteins in early postimplantation embryos suggest distinct and sequential developmental roles
    S Cereghini
    Unité de Génétique de la Différenciation, UA 1149 du CNRS, Institut Pasteur, Paris, France
    Development 116:783-97. 1992
    The homeoproteins HNF1 (LFB1/HNF1-A) and vHNF1 (LFB3/HNF1 beta) interact with an essential control element of a group of liver-specific genes...
  28. ncbi Hepatocyte nuclear factor 1 inactivation results in hepatic dysfunction, phenylketonuria, and renal Fanconi syndrome
    M 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...
  29. ncbi Role of the hepatocyte nuclear factor-1beta (HNF-1beta) C-terminal domain in Pkhd1 (ARPKD) gene transcription and renal cystogenesis
    Thomas 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...
  30. ncbi The mammal-specific Pdx1 Area II enhancer has multiple essential functions in early endocrine-cell specification and postnatal β-cell maturation
    Yu 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...
  31. doi Transcription factors SOX4 and SOX9 cooperatively control development of bile ducts
    Alexis 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. ..
  32. doi The role of tumor necrosis factor alpha in regulating the expression of Tamm-Horsfall Protein (uromodulin) in thick ascending limbs during kidney injury
    Monique 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...
  33. pmc Hnf-1β transcription factor is an early hif-1α-independent marker of epithelial hypoxia and controls renal repair
    Stanislas 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 ..
  34. ncbi Transcriptional regulation of human and mouse organic anion transporter 1 by hepatocyte nuclear factor 1 alpha/beta
    Takami 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 ..
  35. pmc Neurogenin3 cooperates with Foxa2 to autoactivate its own expression
    Miriam 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
    ....
  36. doi Wnt5a signaling mediates biliary differentiation of fetal hepatic stem/progenitor cells in mice
    Kei Kiyohashi
    Department of Gastroenterology and Hepatology, Tokyo, Japan
    Hepatology 57:2502-13. 2013
    ....
  37. pmc Spatiotemporal patterns of multipotentiality in Ptf1a-expressing cells during pancreas organogenesis and injury-induced facultative restoration
    Fong 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...
  38. doi Pancreas-enriched miRNA refines endocrine cell differentiation
    Sharon 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...
  39. pmc A Notch-dependent molecular circuitry initiates pancreatic endocrine and ductal cell differentiation
    Hung 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
    ....
  40. pmc Cardiovascular defects in a mouse model of HOXA1 syndrome
    Nadja 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...
  41. pmc Ngn3(+) endocrine progenitor cells control the fate and morphogenesis of pancreatic ductal epithelium
    Judith 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...
  42. ncbi HNF-1 alpha and HNF-1 beta expression in mouse intestinal crypts
    M 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...
  43. ncbi Abnormal splicing of hepatocyte nuclear factor-1 beta in the renal cysts and diabetes syndrome
    L 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...
  44. pmc Zyxin regulates migration of renal epithelial cells through activation of hepatocyte nuclear factor-1β
    Yun 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. ..
  45. pmc De novo lumen formation and elongation in the developing nephron: a central role for afadin in apical polarity
    Zhufeng Yang
    Department of Medicine, University of Texas Southwestern Medical Center, Dallas, TX 75390, USA
    Development 140:1774-84. 2013
    ....
  46. doi HNF1B controls proximal-intermediate nephron segment identity in vertebrates by regulating Notch signalling components and Irx1/2
    Claire 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 ..
  47. doi Hepatocyte nuclear factor 1β controls nephron tubular development
    Filippo 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 ..
  48. pmc Notch-mediated patterning and cell fate allocation of pancreatic progenitor cells
    Solomon 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...
  49. doi Hepatocyte nuclear factor 4 alpha is related to survival of the condensed mesenchyme in the developing mouse kidney
    Tomonori 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...
  50. ncbi The onecut transcription factor HNF6 is required for normal development of the biliary tract
    Frederic 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...
  51. ncbi Paracrine action of FGF4 during periimplantation development maintains trophectoderm and primitive endoderm
    Sarah 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...
  52. pmc Species-specific differences in the expression of the HNF1A, HNF1B and HNF4A genes
    Lorna W Harries
    Institute of Biomedical and Clinical Sciences, Peninsula Medical School, University of Exeter, Exeter, United Kingdom
    PLoS ONE 4:e7855. 2009
    The HNF1A, HNF1B and HNF4A genes are part of an autoregulatory network in mammalian pancreas, liver, kidney and gut...
  53. pmc Dynamic signaling network for the specification of embryonic pancreas and liver progenitors
    Ewa 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...
  54. doi Notch2 signaling promotes biliary epithelial cell fate specification and tubulogenesis during bile duct development in mice
    Jan 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...
  55. ncbi Quantitative gene expression profiling reveals a fetal hepatic phenotype of murine ES-derived hepatocytes
    Andrea 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...
  56. pmc Intrahepatic bile ducts develop according to a new mode of tubulogenesis regulated by the transcription factor SOX9
    Aline 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)...
  57. doi Hepatocyte nuclear factor 1alpha and beta control terminal differentiation and cell fate commitment in the gut epithelium
    Anna 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...
  58. pmc The mouse alpha-albumin (afamin) promoter is differentially regulated by hepatocyte nuclear factor 1α and hepatocyte nuclear factor 1β
    Hua Liu
    Department of Microbiology, Immunology, and Molecular Genetics, University of Kentucky, Lexington, Kentucky 40536, USA
    DNA Cell Biol 30:137-47. 2011
    ....
  59. pmc The mouse KRAB zinc-finger protein CHATO is required in embryonic-derived tissues to control yolk sac and placenta morphogenesis
    Maho 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...
  60. ncbi Cystic renal neoplasia following conditional inactivation of apc in mouse renal tubular epithelium
    Chao 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...
  61. pmc Sox9-haploinsufficiency causes glucose intolerance in mice
    Claire 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...
  62. pmc Hepatocyte nuclear factor 3beta (Foxa2) is dispensable for maintaining the differentiated state of the adult hepatocyte
    N 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...
  63. ncbi ME1 and GE1: basic helix-loop-helix transcription factors expressed at high levels in the developing nervous system and in morphogenetically active regions
    T Neuman
    Department of Anatomy and Neurobiology, Colorado State University, Fort Collins 80523
    Eur J Neurosci 5:311-8. 1993
    ....
  64. ncbi Localization of sequences related to the human RAD6 DNA repair gene on mouse chromosomes 11 and 13
    M L Roller
    Department of Human Genetics, University of Michigan Medical School, Ann Arbor 48109 0618, USA
    Mamm Genome 6:305-6. 1995
  65. ncbi vHNF1 is expressed in epithelial cells of distinct embryonic origin during development and precedes HNF1 expression
    M O Ott
    Departement de Biologie Moleculaire, Institut Pasteur, Paris, France
    Mech Dev 36:47-58. 1991
    HNF1 (Hepatic Nuclear Factor 1) and vHNF1 are transcriptional regulators containing a highly divergent homeodomain...
  66. doi Sall4 regulates cell fate decision in fetal hepatic stem/progenitor cells
    Tsunekazu 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...
  67. pmc Shifting boundaries of retinoic acid activity control hindbrain segmental gene expression
    Ioan 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...
  68. ncbi The vHNF1 homeodomain protein establishes early rhombomere identity by direct regulation of Kreisler expression
    Florence 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 ..
  69. ncbi Cardiomyocyte-specific Bcl-2 overexpression attenuates ischemia-reperfusion injury, immune response during acute rejection, and graft coronary artery disease
    Masashi 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...
  70. ncbi Visceral endoderm function is regulated by quaking and required for vascular development
    Brenda 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...
  71. ncbi The transcription factor hepatocyte nuclear factor-6 controls the development of pancreatic ducts in the mouse
    Christophe 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...
  72. pmc Role of TIF1alpha as a modulator of embryonic transcription in the mouse zygote
    Maria 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...
  73. pmc Plasticity and expanding complexity of the hepatic transcription factor network during liver development
    Irene 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...
  74. pmc Snail activation disrupts tissue homeostasis and induces fibrosis in the adult kidney
    Agnes 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...
  75. pmc Snail2, a mediator of epithelial-mesenchymal transitions, expressed in progenitor cells of the developing endocrine pancreas
    J 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...
  76. pmc Glomerulocystic kidney disease in mice with a targeted inactivation of Wwtr1
    Zakir 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...
  77. pmc The role for HNF-1beta-targeted collectrin in maintenance of primary cilia and cell polarity in collecting duct cells
    Yanling 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...
  78. pmc The homeobox gene Hhex is essential for proper hepatoblast differentiation and bile duct morphogenesis
    Michael 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 ..
  79. ncbi A genetic screen in zebrafish identifies cilia genes as a principal cause of cystic kidney
    Zhaoxia 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...
  80. ncbi Expression of HNF-4alpha (MODY1), HNF-1beta (MODY5), and HNF-1alpha (MODY3) proteins in the developing mouse pancreas
    Takao 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]...
  81. pmc Morphogenetic competence of HNF4 alpha-deficient mouse hepatic cells
    Graham 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...
  82. ncbi Mapping and promoter sequencing of HNF-1beta gene in diabetes-prone and -resistant mice
    H Ueda
    Department of Geriatric Medicine, Osaka University Medical School, 2 2 Yamadaoka, Suita, 565 0871, Osaka, Japan
    Diabetes Res Clin Pract 53:67-71. 2001
    By 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 ..
  83. ncbi Direct regulation of vHnf1 by retinoic acid signaling and MAF-related factors in the neural tube
    Marie Pouilhe
    INSERM, U784, 46 rue d Ulm, 75230 Paris Cedex 05, France
    Dev Biol 309:344-57. 2007
    The 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 ..
  84. pmc The anterior visceral endoderm of the mouse embryo is established from both preimplantation precursor cells and by de novo gene expression after implantation
    Maria 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...
  85. ncbi Defects in brain patterning and head morphogenesis in the mouse mutant Fused toes
    Isabelle 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...
  86. ncbi Newly identified paralogous groups on mouse chromosomes 5 and 11 reveal the age of a T-box cluster duplication
    I 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...
  87. pmc Complex genetic control of HDL levels in mice in response to an atherogenic diet. Coordinate regulation of HDL levels and bile acid metabolism
    D 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...
  88. pmc Notch signaling controls liver development by regulating biliary differentiation
    Yiwei 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...
  89. pmc HNF-1beta regulates transcription of the PKD modifier gene Kif12
    Yimei 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...
  90. pmc Signaling hierarchy downstream of retinoic acid that independently regulates vascular remodeling and endothelial cell proliferation
    Brenda 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
    ....
  91. pmc Comprehensive search for HNF-1beta-regulated genes in mouse hepatoma cells perturbed by transcription regulatory factor-targeted RNAi
    Taku 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
    ....
  92. ncbi The gene coding for variant hepatic nuclear factor 1 (Tcf-2), maps between the Edp-1 and Erba genes on mouse chromosome 11
    I J Karolyi
    Department of Human Genetics, University of Michigan Medical School, Ann Arbor 48109 0618
    Mamm Genome 3:184-5. 1992
  93. pmc Expression of Groucho/TLE proteins during pancreas development
    Brad 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...
  94. ncbi A vHNF1/TCF2-HNF6 cascade regulates the transcription factor network that controls generation of pancreatic precursor cells
    Aurélie 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...
  95. ncbi Regulation of kidney-specific Ksp-cadherin gene promoter by hepatocyte nuclear factor-1beta
    Yun 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...
  96. ncbi Linkage mapping of murine homolog of the yeast SPT6 gene to MMU11B1
    P W Chiang
    Department of Pediatrics, University of Michigan Medical Center, Ann Arbor 48109 0650, USA
    Mamm Genome 7:459-60. 1996
  97. ncbi Regulation of tissue-specific expression of the human and mouse urate transporter 1 gene by hepatocyte nuclear factor 1 alpha/beta and DNA methylation
    Ryota 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...