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
Products:     Hnf1b

Top Publications

  1. de Simone V, De Magistris L, Lazzaro D, Gerstner J, Monaci P, Nicosia A, et al. LFB3, a heterodimer-forming homeoprotein of the LFB1 family, is expressed in specialized epithelia. EMBO J. 1991;10:1435-43 pubmed
    We have cloned and characterized a mouse cDNA coding for LFB3, a DNA binding protein containing an extra-large homeodomain...
  2. Ueda H, Ikegami H, Kawaguchi Y, Fujisawa T, Yamato E, Shibata M, et al. Genetic analysis of late-onset type 2 diabetes in a mouse model of human complex trait. Diabetes. 1999;48:1168-74 pubmed
    ..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 ..
  3. Sirbu I, Gresh L, Barra J, Duester G. Shifting boundaries of retinoic acid activity control hindbrain segmental gene expression. Development. 2005;132:2611-22 pubmed
    ..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...
  4. Rukstalis J, Habener J. Snail2, a mediator of epithelial-mesenchymal transitions, expressed in progenitor cells of the developing endocrine pancreas. Gene Expr Patterns. 2007;7:471-9 pubmed
    ..EMT-like events appear to be involved in the development of the mammalian pancreas in vivo. ..
  5. Verdeguer F, Le Corre S, Fischer E, Callens C, Garbay S, Doyen A, et al. A mitotic transcriptional switch in polycystic kidney disease. Nat Med. 2010;16:106-10 pubmed publisher
    ..We show here that inactivation of Hnf1b from postnatal day 10 onward does not elicit cystic dilations in tubules after their proliferative morphogenetic ..
  6. Ma Z, Gong Y, Patel V, Karner C, Fischer E, Hiesberger T, et al. Mutations of HNF-1beta inhibit epithelial morphogenesis through dysregulation of SOCS-3. Proc Natl Acad Sci U S A. 2007;104:20386-91 pubmed
    ..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. ..
  7. Horikawa Y, Iwasaki N, Hara M, Furuta H, Hinokio Y, Cockburn B, et al. Mutation in hepatocyte nuclear factor-1 beta gene (TCF2) associated with MODY. Nat Genet. 1997;17:384-5 pubmed
  8. Maestro M, Boj S, Luco R, Pierreux C, Cabedo J, Servitja J, et al. Hnf6 and Tcf2 (MODY5) are linked in a gene network operating in a precursor cell domain of the embryonic pancreas. Hum Mol Genet. 2003;12:3307-14 pubmed
    ..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...
  9. Yoshioka K, Kunitomo M, Yanai K, Shimizu H, Nakasono S, Negishi T, et al. Hepatocyte nuclear factor 1? induced by chemical stress accelerates cell proliferation and increases genomic instability in mouse liver. J Recept Signal Transduct Res. 2011;31:132-8 pubmed publisher
    ..Our data extend the current understanding of the mechanism underlying liver regeneration against chemical stress, and identified HNF1? as a novel regulatory factor in this mechanism and as a potential initiator for carcinogenesis. ..

More Information


  1. Xu E, Perlina A, Vu H, TROTH S, Brennan R, Aslamkhan A, et al. Integrated pathway analysis of rat urine metabolic profiles and kidney transcriptomic profiles to elucidate the systems toxicology of model nephrotoxicants. Chem Res Toxicol. 2008;21:1548-61 pubmed publisher
    ..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.
  2. Pontoglio M, Barra J, Hadchouel M, Doyen A, Kress C, Bach J, et al. Hepatocyte nuclear factor 1 inactivation results in hepatic dysfunction, phenylketonuria, and renal Fanconi syndrome. Cell. 1996;84:575-85 pubmed
    ..The resulting massive urinary glucose loss leads to energy and water wasting. HNF1-deficient mice may provide a model for human renal Fanconi syndrome...
  3. Fischer E, Legue E, Doyen A, Nato F, Nicolas J, Torres V, et al. Defective planar cell polarity in polycystic kidney disease. Nat Genet. 2006;38:21-3 pubmed
    ..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. ..
  4. Edghill E, Bingham C, Ellard S, Hattersley A. Mutations in hepatocyte nuclear factor-1beta and their related phenotypes. J Med Genet. 2006;43:84-90 pubmed
    ..Molecular genetic testing for HNF-1beta mutations should be considered in patients with unexplained renal cysts (including GCKD), especially when associated with diabetes, early-onset gout, or uterine abnormalities. ..
  5. Cereghini S, Ott M, Power S, Maury M. Expression patterns of vHNF1 and HNF1 homeoproteins in early postimplantation embryos suggest distinct and sequential developmental roles. Development. 1992;116:783-97 pubmed
    The homeoproteins HNF1 (LFB1/HNF1-A) and vHNF1 (LFB3/HNF1 beta) interact with an essential control element of a group of liver-specific genes...
  6. Oliver Krasinski J, Kasner M, Yang J, Crutchlow M, Rustgi A, Kaestner K, et al. The diabetes gene Pdx1 regulates the transcriptional network of pancreatic endocrine progenitor cells in mice. J Clin Invest. 2009;119:1888-98 pubmed publisher
    ..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...
  7. Haumaitre C, Barbacci E, Jenny M, Ott M, Gradwohl G, Cereghini S. Lack of TCF2/vHNF1 in mice leads to pancreas agenesis. Proc Natl Acad Sci U S A. 2005;102:1490-5 pubmed
    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...
  8. Hiesberger T, Shao X, Gourley E, Reimann A, Pontoglio M, Igarashi P. Role of the hepatocyte nuclear factor-1beta (HNF-1beta) C-terminal domain in Pkhd1 (ARPKD) gene transcription and renal cystogenesis. J Biol Chem. 2005;280:10578-86 pubmed
    ..Expression of HNF-1alpha in proximal tubules may protect against cystogenesis. ..
  9. Barbacci E, Chalkiadaki A, Masdeu C, Haumaitre C, Lokmane L, Loirat C, et al. HNF1beta/TCF2 mutations impair transactivation potential through altered co-regulator recruitment. Hum Mol Genet. 2004;13:3139-49 pubmed
    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 ..
  10. Hiesberger T, Bai Y, Shao X, McNally B, Sinclair A, Tian X, et al. Mutation of hepatocyte nuclear factor-1beta inhibits Pkhd1 gene expression and produces renal cysts in mice. J Clin Invest. 2004;113:814-25 pubmed
    ..These studies identify a link between two cystic disease genes, HNF1beta (MODY5) and PKHD1 (ARPKD)...
  11. Gresh L, Fischer E, Reimann A, Tanguy M, Garbay S, Shao X, et al. A transcriptional network in polycystic kidney disease. EMBO J. 2004;23:1657-68 pubmed
    ..Hepatocyte Nuclear Factor 1beta (HNF1beta) is a homeoprotein predominantly expressed in renal, pancreatic and hepatic epithelia...
  12. Kopp J, Dubois C, Schaffer A, Hao E, Shih H, Seymour P, et al. Sox9+ ductal cells are multipotent progenitors throughout development but do not produce new endocrine cells in the normal or injured adult pancreas. Development. 2011;138:653-65 pubmed publisher
  13. Solar M, Cardalda C, Houbracken I, Martin M, Maestro M, De Medts N, et al. Pancreatic exocrine duct cells give rise to insulin-producing beta cells during embryogenesis but not after birth. Dev Cell. 2009;17:849-60 pubmed publisher
    ..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 ..
  14. Lokmane L, Heliot C, Garcia Villalba P, Fabre M, Cereghini S. vHNF1 functions in distinct regulatory circuits to control ureteric bud branching and early nephrogenesis. Development. 2010;137:347-57 pubmed publisher
    ..Here, we identify the transcription factor vHNF1 (HNF1beta) as a crucial regulator of these early developmental events...
  15. Coffinier C, Thepot D, Babinet C, Yaniv M, Barra J. Essential role for the homeoprotein vHNF1/HNF1beta in visceral endoderm differentiation. Development. 1999;126:4785-94 pubmed
    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...
  16. Barbacci E, Reber M, Ott M, Breillat C, Huetz F, Cereghini S. Variant hepatocyte nuclear factor 1 is required for visceral endoderm specification. Development. 1999;126:4795-805 pubmed
    ..We show that variant Hepatocyte Nuclear Factor 1 (vHNF1), a homeodomain-containing transcription factor first expressed in the primitive endoderm, is required for the ..
  17. Yang Z, Zimmerman S, BRAKEMAN P, Beaudoin G, Reichardt L, MARCIANO D. De novo lumen formation and elongation in the developing nephron: a central role for afadin in apical polarity. Development. 2013;140:1774-84 pubmed publisher
  18. Ott M, Rey Campos J, Cereghini S, Yaniv M. vHNF1 is expressed in epithelial cells of distinct embryonic origin during development and precedes HNF1 expression. Mech Dev. 1991;36:47-58 pubmed
    HNF1 (Hepatic Nuclear Factor 1) and vHNF1 are transcriptional regulators containing a highly divergent homeodomain...
  19. Desgrange A, Heliot C, Skovorodkin I, Akram S, Heikkilä J, Ronkainen V, et al. HNF1B controls epithelial organization and cell polarity during ureteric bud branching and collecting duct morphogenesis. Development. 2017;144:4704-4719 pubmed publisher
    ..The transcription factor HNF1B is required for the early steps of ureteric bud branching, yet the molecular and cellular events regulated by ..
  20. Kanazawa T, Konno A, Hashimoto Y, Kon Y. Hepatocyte nuclear factor 4 alpha is related to survival of the condensed mesenchyme in the developing mouse kidney. Dev Dyn. 2010;239:1145-54 pubmed publisher
    ..These results suggest the possibility that Hnf4alpha plays an important role in the regulation of the cell survival at the CM stage in nephrogenesis. ..
  21. Jochheim A, Hillemann T, Kania G, Scharf J, Attaran M, Manns M, et al. Quantitative gene expression profiling reveals a fetal hepatic phenotype of murine ES-derived hepatocytes. Int J Dev Biol. 2004;48:23-9 pubmed
    ..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. ..
  22. Ueda H, Ikegami H, Kawaguchi Y, Fujisawa T, Nojima K, Babaya N, et al. Mapping and promoter sequencing of HNF-1beta gene in diabetes-prone and -resistant mice. Diabetes Res Clin Pract. 2001;53:67-71 pubmed
    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 ..
  23. Tanaka M, Nakae S, Terry R, Mokhtari G, Gunawan F, Balsam L, et al. Cardiomyocyte-specific Bcl-2 overexpression attenuates ischemia-reperfusion injury, immune response during acute rejection, and graft coronary artery disease. Blood. 2004;104:3789-96 pubmed
    ..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. ..
  24. Wu H, Yu W, Meng F, Mi J, Peng J, Liu J, et al. Polychlorinated biphenyls-153 induces metabolic dysfunction through activation of ROS/NF-κB signaling via downregulation of HNF1b. Redox Biol. 2017;12:300-310 pubmed publisher
    ..PCB-153 induced oxidative stress and decreased hepatocyte nuclear factor 1b (HNF1b) and glutathione peroxidase 1 (GPx1) expression in vivo and in vitro...
  25. Kiyohashi K, Kakinuma S, Kamiya A, Sakamoto N, Nitta S, Yamanaka H, et al. Wnt5a signaling mediates biliary differentiation of fetal hepatic stem/progenitor cells in mice. Hepatology. 2013;57:2502-13 pubmed publisher
    ..Wnt5a-mediated signaling in fetal hepatic stem/progenitor cells suppresses biliary differentiation. These findings also suggest that activation of CaMKII by Wnt5a signaling suppresses biliary differentiation. (HEPATOLOGY 2013;). ..
  26. Kim F, Sing l A, Kaneko T, Bieman M, Stallwood N, Sadl V, et al. The vHNF1 homeodomain protein establishes early rhombomere identity by direct regulation of Kreisler expression. Mech Dev. 2005;122:1300-9 pubmed
    ..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 ..
  27. Lakhia R, Hajarnis S, Williams D, Aboudehen K, Yheskel M, Xing C, et al. MicroRNA-21 Aggravates Cyst Growth in a Model of Polycystic Kidney Disease. J Am Soc Nephrol. 2016;27:2319-30 pubmed publisher
    ..Our results suggest that inhibiting miR-21 is a potential new therapeutic approach to slow cyst growth in PKD. ..
  28. Poncy A, Antoniou A, Cordi S, Pierreux C, Jacquemin P, Lemaigre F. Transcription factors SOX4 and SOX9 cooperatively control development of bile ducts. Dev Biol. 2015;404:136-48 pubmed publisher
    ..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. ..
  29. Patel V, Williams D, Hajarnis S, Hunter R, Pontoglio M, Somlo S, et al. miR-17~92 miRNA cluster promotes kidney cyst growth in polycystic kidney disease. Proc Natl Acad Sci U S A. 2013;110:10765-70 pubmed publisher
    ..Our results also provide a unique hypothesis for disease progression in PKD involving miRNAs and regulation of PKD gene dosage. ..
  30. Qian C, Knol J, Igarashi P, Lin F, Zylstra U, Teh B, et al. Cystic renal neoplasia following conditional inactivation of apc in mouse renal tubular epithelium. J Biol Chem. 2005;280:3938-45 pubmed
    ..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. ..
  31. Liu H, Ren H, Spear B. The mouse alpha-albumin (afamin) promoter is differentially regulated by hepatocyte nuclear factor 1? and hepatocyte nuclear factor 1?. DNA Cell Biol. 2011;30:137-47 pubmed publisher
  32. Dubois C, Shih H, Seymour P, Patel N, Behrmann J, Ngo V, et al. Sox9-haploinsufficiency causes glucose intolerance in mice. PLoS ONE. 2011;6:e23131 pubmed publisher has been suggested that Sox9 controls expression of a network of important developmental regulators, including Tcf2/MODY5, Hnf6, and Foxa2, in pancreatic progenitor cells...
  33. Bai Y, Pontoglio M, Hiesberger T, Sinclair A, Igarashi P. Regulation of kidney-specific Ksp-cadherin gene promoter by hepatocyte nuclear factor-1beta. Am J Physiol Renal Physiol. 2002;283:F839-51 pubmed
    ..Mutations of HNF-1beta, as occur in humans with inherited renal cysts and diabetes, may cause dysregulated Ksp-cadherin promoter activity. ..
  34. Faguer S, Mayeur N, Casemayou A, Pageaud A, Courtellemont C, Cartery C, et al. Hnf-1? transcription factor is an early hif-1?-independent marker of epithelial hypoxia and controls renal repair. PLoS ONE. 2013;8:e63585 pubmed publisher
    ..In addition, early decrease in Hnf1b expression is associated with the transient over-expression of one of its main target genes, the suppressor of ..
  35. Wandzioch E, Zaret K. Dynamic signaling network for the specification of embryonic pancreas and liver progenitors. Science. 2009;324:1707-10 pubmed publisher
    ..These findings may enhance progenitor cell specification from stem cells for biomedical purposes and can help explain incomplete programming in stem cell differentiation protocols. ..
  36. Pouilhe M, Gilardi Hebenstreit P, Desmarquet Trin Dinh C, Charnay P. Direct regulation of vHnf1 by retinoic acid signaling and MAF-related factors in the neural tube. Dev Biol. 2007;309:344-57 pubmed
    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 ..
  37. Ruvinsky I, Silver L. Newly identified paralogous groups on mouse chromosomes 5 and 11 reveal the age of a T-box cluster duplication. Genomics. 1997;40:262-6 pubmed
    ..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. ..
  38. Aboudehen K, Noureddine L, Cobo Stark P, Avdulov S, Farahani S, Gearhart M, et al. Hepatocyte Nuclear Factor-1β Regulates Urinary Concentration and Response to Hypertonicity. J Am Soc Nephrol. 2017;28:2887-2900 pubmed publisher
    ..These findings reveal a novel role of HNF-1β in osmoregulation and identify multiple mechanisms, whereby mutations of HNF-1β produce defects in urinary concentration. ..
  39. Li Y, Liu J, McLaughlin N, Bachvarov D, Saifudeen Z, El Dahr S. Genome-wide analysis of the p53 gene regulatory network in the developing mouse kidney. Physiol Genomics. 2013;45:948-64 pubmed publisher
    ..We conclude p53 targets transcriptional networks regulating nephrogenesis and cellular metabolism during kidney development. ..
  40. Tanaka T, Tomaru Y, Nomura Y, Miura H, Suzuki M, Hayashizaki Y. Comprehensive search for HNF-1beta-regulated genes in mouse hepatoma cells perturbed by transcription regulatory factor-targeted RNAi. Nucleic Acids Res. 2004;32:2740-50 pubmed
  41. Smith A, Radice G, Lang R. Which FGF ligands are involved in lens induction?. Dev Biol. 2010;337:195-8 pubmed publisher
  42. Machleder D, Ivandic B, Welch C, Castellani L, Reue K, Lusis A. Complex genetic control of HDL levels in mice in response to an atherogenic diet. Coordinate regulation of HDL levels and bile acid metabolism. J Clin Invest. 1997;99:1406-19 pubmed
    ..Furthermore, we observed that two of the loci for C7AH expression contributed to differences in gallstone formation between these strains. ..
  43. Roller M, Lossie A, Koken M, Smit E, Hagemeijer A, Camper S. Localization of sequences related to the human RAD6 DNA repair gene on mouse chromosomes 11 and 13. Mamm Genome. 1995;6:305-6 pubmed
  44. Torres Padilla M, Zernicka Goetz M. Role of TIF1alpha as a modulator of embryonic transcription in the mouse zygote. J Cell Biol. 2006;174:329-38 pubmed
    ..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. ..
  45. Yahoo N, Pournasr B, Rostamzadeh J, Hakhamaneshi M, Ebadifar A, Fathi F, et al. Forced expression of Hnf1b/Foxa3 promotes hepatic fate of embryonic stem cells. Biochem Biophys Res Commun. 2016;474:199-205 pubmed publisher
    ..We screened the effects of candidate transcription factors Hnf1b, Hnf1a, Hnf4a, Foxa1, Foxa3 and Hex, and determined that the combination of Hnf1b/Foxa3 promoted expression of ..
  46. Clotman F, Lannoy V, Reber M, Cereghini S, Cassiman D, Jacquemin P, et al. The onecut transcription factor HNF6 is required for normal development of the biliary tract. Development. 2002;129:1819-28 pubmed
    ..The morphology of the intrahepatic bile ducts was identical to that seen in mice whose Hnf1beta gene has been conditionally inactivated in the liver...
  47. Shibata M, GARCIA GARCIA M. The mouse KRAB zinc-finger protein CHATO is required in embryonic-derived tissues to control yolk sac and placenta morphogenesis. Dev Biol. 2011;349:331-41 pubmed publisher
    ..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...
  48. Bohnsack B, Lai L, Northrop J, Justice M, Hirschi K. Visceral endoderm function is regulated by quaking and required for vascular development. Genesis. 2006;44:93-104 pubmed
    ..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. ..
  49. Delgado I, Carrasco M, Cano E, Carmona R, Garcia Carbonero R, Marín Gómez L, et al. GATA4 loss in the septum transversum mesenchyme promotes liver fibrosis in mice. Hepatology. 2014;59:2358-70 pubmed publisher
    ..Haploinsufficiency of Gata4 accelerated CCl4 -induced liver fibrosis in adult mice. Moreover, Gata4 expression was dramatically reduced in advanced hepatic fibrosis and cirrhosis in humans...
  50. Hajarnis S, Patel V, Aboudehen K, Attanasio M, Cobo Stark P, Pontoglio M, et al. Transcription Factor Hepatocyte Nuclear Factor-1β (HNF-1β) Regulates MicroRNA-200 Expression through a Long Noncoding RNA. J Biol Chem. 2015;290:24793-805 pubmed publisher
    ..These studies reveal a novel pathway whereby HNF-1β directly contributes to the control of miRNAs that are involved in epithelial-mesenchymal transition and cystic kidney disease. ..
  51. Goldin S, Papaioannou V. Paracrine action of FGF4 during periimplantation development maintains trophectoderm and primitive endoderm. Genesis. 2003;36:40-7 pubmed
    ..Thus, postimplantation lethality of Fgf4(-/-) embryos likely results from the failure of proper differentiation and function of extraembryonic cell types. ..
  52. Kodama S, Nakano Y, Hirata K, Furuyama K, Horiguchi M, Kuhara T, et al. Diabetes Caused by Elastase-Cre-Mediated Pdx1 Inactivation in Mice. Sci Rep. 2016;6:21211 pubmed publisher
  53. Coffinier C, Gresh L, Fiette L, Tronche F, Schutz G, Babinet C, et al. Bile system morphogenesis defects and liver dysfunction upon targeted deletion of HNF1beta. Development. 2002;129:1829-38 pubmed
    The inactivation of the Hnf1beta gene identified an essential role in epithelial differentiation of the visceral endoderm and resulted in early embryonic death...
  54. Makki N, Capecchi M. Cardiovascular defects in a mouse model of HOXA1 syndrome. Hum Mol Genet. 2012;21:26-31 pubmed publisher
    ..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. ..
  55. Kredo Russo S, Mandelbaum A, Ness A, Alon I, Lennox K, Behlke M, et al. Pancreas-enriched miRNA refines endocrine cell differentiation. Development. 2012;139:3021-31 pubmed publisher
    ..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...
  56. Hossain Z, Ali S, Ko H, Xu J, Ng C, Guo K, et al. Glomerulocystic kidney disease in mice with a targeted inactivation of Wwtr1. Proc Natl Acad Sci U S A. 2007;104:1631-6 pubmed
    ..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. ..
  57. Kornfeld J, Baitzel C, Könner A, Nicholls H, Vogt M, Herrmanns K, et al. Obesity-induced overexpression of miR-802 impairs glucose metabolism through silencing of Hnf1b. Nature. 2013;494:111-5 pubmed publisher
    ..We identify Hnf1b (also known as Tcf2) as a target of miR-802-dependent silencing, and show that short hairpin RNA (shRNA)-mediated ..
  58. Harries L, Brown J, Gloyn A. Species-specific differences in the expression of the HNF1A, HNF1B and HNF4A genes. PLoS ONE. 2009;4:e7855 pubmed publisher
    The HNF1A, HNF1B and HNF4A genes are part of an autoregulatory network in mammalian pancreas, liver, kidney and gut...
  59. Heitmeier M, McCracken R, Micanovic R, Khan S, El Achkar T. The role of tumor necrosis factor alpha in regulating the expression of Tamm-Horsfall Protein (uromodulin) in thick ascending limbs during kidney injury. Am J Nephrol. 2014;40:458-67 pubmed publisher
    ..We propose that TNFα regulates THP expression in a homeostatic setting, but the impact of TNFα on THP during kidney injury is superseded by other factors that could inhibit HNF1β-mediated expression of THP. ..
  60. Hofmann J, Zovein A, Koh H, Radtke F, Weinmaster G, Iruela Arispe M. Jagged1 in the portal vein mesenchyme regulates intrahepatic bile duct development: insights into Alagille syndrome. Development. 2010;137:4061-72 pubmed publisher
    ..These findings uncover the cellular basis for the defining feature of AGS, identify mesenchymal Jag1-dependent and -independent stages of duct development, and provide mechanistic information for the role of Jag1 in IHBD formation...
  61. Poll A, Pierreux C, Lokmane L, Haumaitre C, Achouri Y, Jacquemin P, et al. A vHNF1/TCF2-HNF6 cascade regulates the transcription factor network that controls generation of pancreatic precursor cells. Diabetes. 2006;55:61-9 pubmed
    ..Among the latter, variant HNF1 (vHNF1)/TCF2, which is expressed before HNF6 in the endoderm, was found to be critical for HNF6 expression...
  62. Nagy I, Xu Q, Naillat F, Ali N, Miinalainen I, Samoylenko A, et al. Impairment of Wnt11 function leads to kidney tubular abnormalities and secondary glomerular cystogenesis. BMC Dev Biol. 2016;16:30 pubmed publisher
    ..The C57Bl6 Wnt11 (-/-) mouse generated here provides a model for studying the mechanisms behind tubular anomalies and glomerular cyst formation. ..
  63. Afelik S, Pool B, Schmerr M, Penton C, Jensen J. Wnt7b is required for epithelial progenitor growth and operates during epithelial-to-mesenchymal signaling in pancreatic development. Dev Biol. 2015;399:204-17 pubmed publisher
    ..We conclude that Wnt7b helps coordinate pancreatic development through autocrine, as well as paracrine mechanisms, and as such represents a novel bi-modal morphogen ligand. ..
  64. Yang Y, Magnuson M, Stein R, Wright C. The mammal-specific Pdx1 Area II enhancer has multiple essential functions in early endocrine cell specification and postnatal β-cell maturation. Development. 2017;144:248-257 pubmed publisher
    ..Therefore, trans-regulatory integration through Area II mediates a surprisingly extensive range of progenitor and β-cell-specific Pdx1 functions. ..
  65. Gong Y, Ma Z, Patel V, Fischer E, Hiesberger T, Pontoglio M, et al. HNF-1beta regulates transcription of the PKD modifier gene Kif12. J Am Soc Nephrol. 2009;20:41-7 pubmed publisher
    ..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. ..
  66. Harries L, Ellard S, Jones R, Hattersley A, Bingham C. Abnormal splicing of hepatocyte nuclear factor-1 beta in the renal cysts and diabetes syndrome. Diabetologia. 2004;47:937-42 pubmed
    ..This is the first study to define the pathogenic consequences of mutations within the HNF-1 beta gene by mRNA analysis. This type of approach is a useful and important tool to define mutational mechanisms and determine pathogenicity. ..
  67. Afelik S, Qu X, Hasrouni E, Bukys M, Deering T, Nieuwoudt S, et al. Notch-mediated patterning and cell fate allocation of pancreatic progenitor cells. Development. 2012;139:1744-53 pubmed publisher
    ..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-j? to the Nkx6.1 proximal promoter. ..
  68. Magenheim J, Klein A, Stanger B, Ashery Padan R, Sosa Pineda B, Gu G, et al. Ngn3(+) endocrine progenitor cells control the fate and morphogenesis of pancreatic ductal epithelium. Dev Biol. 2011;359:26-36 pubmed publisher
    ..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. ..
  69. Krupczak Hollis K, Wang X, Kalinichenko V, Gusarova G, Wang I, Dennewitz M, et al. The mouse Forkhead Box m1 transcription factor is essential for hepatoblast mitosis and development of intrahepatic bile ducts and vessels during liver morphogenesis. Dev Biol. 2004;276:74-88 pubmed
  70. Saji T, Kikuchi R, Kusuhara H, Kim I, Gonzalez F, Sugiyama Y. Transcriptional regulation of human and mouse organic anion transporter 1 by hepatocyte nuclear factor 1 alpha/beta. J Pharmacol Exp Ther. 2008;324:784-90 pubmed
    ..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 ..
  71. Pierreux C, Poll A, Kemp C, Clotman F, Maestro M, Cordi S, et al. The transcription factor hepatocyte nuclear factor-6 controls the development of pancreatic ducts in the mouse. Gastroenterology. 2006;130:532-41 pubmed
    ..HNF-6 controls a network of genes involved in cilium formation and in hereditary polycystic diseases. Finally, HNF-6 deficiency represents a genetically defined model of pancreatic cystic disease. ..
  72. Torres Padilla M, Richardson L, Kolasinska P, Meilhac S, Luetke Eversloh M, Zernicka Goetz M. The anterior visceral endoderm of the mouse embryo is established from both preimplantation precursor cells and by de novo gene expression after implantation. Dev Biol. 2007;309:97-112 pubmed
    ..Together, these results suggest that the orientation of the anterior-posterior axis is already anticipated before AVE migration. ..
  73. Choi Y, McNally B, Igarashi P. Zyxin regulates migration of renal epithelial cells through activation of hepatocyte nuclear factor-1?. Am J Physiol Renal Physiol. 2013;305:F100-10 pubmed publisher
    ..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. ..
  74. Pan F, Bankaitis E, Boyer D, Xu X, Van De Casteele M, Magnuson M, et al. Spatiotemporal patterns of multipotentiality in Ptf1a-expressing cells during pancreas organogenesis and injury-induced facultative restoration. Development. 2013;140:751-64 pubmed publisher
    ..Thus, we show that acinar cells, without exogenously introduced factors, can regain aspects of embryonic multipotentiality under injury, and convert into mature ?-cells...
  75. Karolyi I, Guenet J, Rey Campos J, Camper S. The gene coding for variant hepatic nuclear factor 1 (Tcf-2), maps between the Edp-1 and Erba genes on mouse chromosome 11. Mamm Genome. 1992;3:184-5 pubmed