Gene Symbol: hnf1ba
Description: HNF1 homeobox Ba
Alias: chunp6877, hnf1b, mst, tcf2, vhnf1, hepatocyte nuclear factor 1-beta-A, HNF-1-beta-A, HNF-1B-2, HNF-1B-A, HNF1 homeobox b, TCF-2, hepatocyte nuclear factor 1-beta, hepatocyte nuclear factor 1-beta-2, hob, transcription factor 2, hepatic, variant hepatic nuclear factor 1
Species: zebrafish
Products:     hnf1ba

Top Publications

  1. Wiellette E, Sive H. vhnf1 and Fgf signals synergize to specify rhombomere identity in the zebrafish hindbrain. Development. 2003;130:3821-9 pubmed
    ..for fibroblast growth factor (Fgf) signaling and for the transcription factor variant hepatocyte nuclear factor 1 (vhnf1) in specification of rhombomeres 5 and 6 (r5+r6)...
  2. Choe S, SAGERSTROM C. Paralog group 1 hox genes regulate rhombomere 5/6 expression of vhnf1, a repressor of rostral hindbrain fates, in a meis-dependent manner. Dev Biol. 2004;271:350-61 pubmed
    ..r4 transformation of the caudal hindbrain is mediated by paralog group 1 (PG1) hox genes and can be repressed by vhnf1, a gene expressed in r5-r6...
  3. Bagnat M, Cheung I, Mostov K, Stainier D. Genetic control of single lumen formation in the zebrafish gut. Nat Cell Biol. 2007;9:954-60 pubmed
    ..Here we show that zebrafish mutants for the MODY5 and familial GCKD gene tcf2 (also known as vhnf1) fail to specify a single lumen in their gut tube and instead develop multiple lumens...
  4. Maves L, Kimmel C. Dynamic and sequential patterning of the zebrafish posterior hindbrain by retinoic acid. Dev Biol. 2005;285:593-605 pubmed
    ..identity of each rhombomere is specified by the expression of conserved transcription factors, including Krox-20, vHnf1, Val (Kreisler, Mafb) and several Hox proteins...
  5. Hernandez R, Putzke A, Myers J, Margaretha L, Moens C. Cyp26 enzymes generate the retinoic acid response pattern necessary for hindbrain development. Development. 2007;134:177-87 pubmed
    ..We present a ;gradient-free' model for hindbrain patterning in which differential RA responsiveness along the hindbrain anterior-posterior axis is shaped primarily by the dynamic expression of RA-degrading enzymes. ..
  6. Gongal P, Waskiewicz A. Zebrafish model of holoprosencephaly demonstrates a key role for TGIF in regulating retinoic acid metabolism. Hum Mol Genet. 2008;17:525-38 pubmed
    ..The consequences of abnormal RA levels for forebrain patterning are profound, and imply that in human patients with TGIF deficiencies, increased forebrain RA levels contribute to the development of HPE. ..
  7. Sun Z, Hopkins N. vhnf1, the MODY5 and familial GCKD-associated gene, regulates regional specification of the zebrafish gut, pronephros, and hindbrain. Genes Dev. 2001;15:3217-29 pubmed
    Mutations in the homeobox gene vHnf1 are associated with human diseases MODY5 (maturity-onset diabetes of the young, type V) and familial GCKD (glomerulocystic kidney disease)...
  8. Gongal P, March L, Holly V, Pillay L, Berry Wynne K, Kagechika H, et al. Hmx4 regulates Sonic hedgehog signaling through control of retinoic acid synthesis during forebrain patterning. Dev Biol. 2011;355:55-64 pubmed publisher
    ..We propose that Hmx4 is a critical regulator of retinoic acid synthesis in a developing embryo, and that this regulation is essential for controlling Shh signaling and forebrain development. ..
  9. Hernandez R, Rikhof H, Bachmann R, Moens C. vhnf1 integrates global RA patterning and local FGF signals to direct posterior hindbrain development in zebrafish. Development. 2004;131:4511-20 pubmed
    ..the paraxial mesoderm; fibroblast growth factor (Fgf) signals from r4; and variant hepatocyte nuclear factor 1 (vhnf1, also known as tcf2), a homeodomain transcription factor expressed posterior to the r4-5 boundary...

More Information


  1. Matthews R, Eauclaire S, Mugnier M, Lorent K, Cui S, Ross M, et al. DNA hypomethylation causes bile duct defects in zebrafish and is a distinguishing feature of infantile biliary atresia. Hepatology. 2011;53:905-14 pubmed publisher
  2. Cui S, Eauclaire S, Matthews R. Interferon-gamma directly mediates developmental biliary defects. Zebrafish. 2013;10:177-83 pubmed publisher
    ..defects are associated with decreased cholangiocyte proliferation and with a decrease in the expression of vhnf1 (hnf1b, tcf2), which encodes a homeodomain protein with previously reported roles in biliary development in multiple ..
  3. Cui S, Erlichman J, Russo P, Haber B, Matthews R. Intrahepatic biliary anomalies in a patient with Mowat-Wilson syndrome uncover a role for the zinc finger homeobox gene zfhx1b in vertebrate biliary development. J Pediatr Gastroenterol Nutr. 2011;52:339-44 pubmed publisher
    ..These findings were associated with decreased expression of vhnf1, a transcription factor known to be important in biliary development in zebrafish and in mammals...
  4. Chi N, Shaw R, Jungblut B, Huisken J, Ferrer T, Arnaout R, et al. Genetic and physiologic dissection of the vertebrate cardiac conduction system. PLoS Biol. 2008;6:e109 pubmed publisher
    ..Positional cloning of hobgoblin(s634) revealed that tcf2, a homeobox transcription factor gene involved in mature onset diabetes of the young and familial glomerulocystic ..
  5. Xu L, Feng Z, Sinha D, Ducos B, Ebenstein Y, Tadmor A, et al. Spatiotemporal manipulation of retinoic acid activity in zebrafish hindbrain development via photo-isomerization. Development. 2012;139:3355-62 pubmed publisher
  6. McGonnell I, Fowkes R. Fishing for gene function--endocrine modelling in the zebrafish. J Endocrinol. 2006;189:425-39 pubmed
    ..We anticipate that the use of these techniques will make the zebrafish a prominent model in endocrine research in the coming years. ..
  7. Tang V, COFER Z, Cui S, Sapp V, Loomes K, Matthews R. Loss of a Candidate Biliary Atresia Susceptibility Gene, add3a, Causes Biliary Developmental Defects in Zebrafish. J Pediatr Gastroenterol Nutr. 2016;63:524-530 pubmed
    ..Our results support previous studies identifying ADD3 as a putative genetic risk factor for BA susceptibility. Our results also provide evidence that add3a may be affecting the Hh pathway, an important factor in BA pathogenesis. ..
  8. Naylor R, Przepiorski A, Ren Q, Yu J, Davidson A. HNF1? is essential for nephron segmentation during nephrogenesis. J Am Soc Nephrol. 2013;24:77-87 pubmed publisher
    ..Using zebrafish, we found that the HNF1? paralogues hnf1ba and hnf1bb, which encode homeodomain transcription factors, are essential for normal segmentation of nephrons...
  9. Lancman J, Zvenigorodsky N, Gates K, Zhang D, Solomon K, Humphrey R, et al. Specification of hepatopancreas progenitors in zebrafish by hnf1ba and wnt2bb. Development. 2013;140:2669-79 pubmed publisher
    ..Using a novel hypomorphic hnf1ba zebrafish mutant that exhibits pancreas hypoplasia, as observed in HNF1B monogenic diabetes, we show that hnf1ba ..
  10. Naylor R, Dodd R, Davidson A. Caudal migration and proliferation of renal progenitors regulates early nephron segment size in zebrafish. Sci Rep. 2016;6:35647 pubmed publisher
    ..Together, these data provide new insights into early nephron morphogenesis and demonstrate the importance of cell movement and proliferation in determining initial nephron segment size. ..
  11. Kim H, Sumanas S, Palencia Desai S, Dong Y, Chen J, Lin S. Genetic analysis of early endocrine pancreas formation in zebrafish. Mol Endocrinol. 2006;20:194-203 pubmed
    ..Further study on these mutants will reveal important information on novel genes involved in pancreatic islet cell specification and morphogenesis. ..
  12. Nakamura M, Choe S, Runko A, Gardner P, SAGERSTROM C. Nlz1/Znf703 acts as a repressor of transcription. BMC Dev Biol. 2008;8:108 pubmed publisher
    ..We conclude that Nlz1/Znf703 acts as a repressor of transcription and hypothesize that other NET family members function in a similar manner. ..
  13. Tehrani Z, Lin S. Antagonistic interactions of hedgehog, Bmp and retinoic acid signals control zebrafish endocrine pancreas development. Development. 2011;138:631-40 pubmed publisher
    ..These results identify multiple sequential roles for Hh in pancreas development and highlight an unexpected antagonistic relationship between Hh and other signaling pathways to control pancreatic specification and differentiation. ..
  14. Drummond D, Cheng C, Selland L, Hocking J, Prichard L, Waskiewicz A. The role of Zic transcription factors in regulating hindbrain retinoic acid signaling. BMC Dev Biol. 2013;13:31 pubmed publisher
    ..Zic transcription factors function in patterning hindbrain motor neurons through their regulation of embryonic retinoic acid signaling. ..
  15. Her G, Cheng C, Hong J, Sundaram G, Wu J. Imbalance in liver homeostasis leading to hyperplasia by overexpressing either one of the Bcl-2-related genes, zfBLP1 and zfMcl-1a. Dev Dyn. 2006;235:515-23 pubmed
  16. Choe S, Hirsch N, Zhang X, SAGERSTROM C. hnf1b genes in zebrafish hindbrain development. Zebrafish. 2008;5:179-87 pubmed publisher
    The Hnf1b transcription factor acts during formation of rhombomeres (r) 5 and 6 in the hindbrain. To determine if hnf1b is absolutely required in r5/r6, we examined the hnf1b(hi2169) and hnf1b(hi1843) retroviral insertion alleles...
  17. Sukardi H, Zhang X, Lui E, Ung C, Mathavan S, Gong Z, et al. Liver X receptor agonist T0901317 induced liver perturbation in zebrafish: histological, gene set enrichment and expression analyses. Biochim Biophys Acta. 2012;1820:33-43 pubmed publisher
    ..Our study underscores the potential of using zebrafish model coupled with transcriptomic analysis to capture pharmacological and toxicological or pathological events induced by LXR modulators. ..
  18. Hans S, Westerfield M. Changes in retinoic acid signaling alter otic patterning. Development. 2007;134:2449-58 pubmed
    ..fgf3;tcf2 double mutants form otic vesicles similar to RA-signaling-depleted embryos, suggesting a signal from rhombomere 5-6 ..
  19. Roy N, SAGERSTROM C. An early Fgf signal required for gene expression in the zebrafish hindbrain primordium. Brain Res Dev Brain Res. 2004;148:27-42 pubmed
    ..Our results demonstrate an early role for Fgf signaling and reveal a dynamic relationship between the RA and Fgf signaling pathways during hindbrain development. ..
  20. Rai K, Nadauld L, Chidester S, Manos E, James S, Karpf A, et al. Zebra fish Dnmt1 and Suv39h1 regulate organ-specific terminal differentiation during development. Mol Cell Biol. 2006;26:7077-85 pubmed
    ..Our results suggest that Dnmt1 activity helps direct histone methylation by Suv39h1 and that, together, Dnmt1 and Suv39h1 help guide the terminal differentiation of particular tissues. ..
  21. Cui S, Capecci L, Matthews R. Disruption of planar cell polarity activity leads to developmental biliary defects. Dev Biol. 2011;351:229-41 pubmed publisher
    ..Knockdown of pk1a led to decreased expression of vhnf1, a homeodomain gene previously shown to be involved in biliary development and in kidney cyst formation; forced ..
  22. Lecaudey V, Anselme I, Rosa F, Schneider Maunoury S. The zebrafish Iroquois gene iro7 positions the r4/r5 boundary and controls neurogenesis in the rostral hindbrain. Development. 2004;131:3121-31 pubmed
    ..This territory is strictly complementary to the expression domain of another homeobox gene, vhnf1, in the caudal neural plate...
  23. Lecaudey V, Ulloa E, Anselme I, Stedman A, Schneider Maunoury S, Pujades C. Role of the hindbrain in patterning the otic vesicle: a study of the zebrafish vhnf1 mutant. Dev Biol. 2007;303:134-43 pubmed
    ..signals in instructing otic vesicle regionalisation, we analysed ear development in zebrafish mutants for vhnf1, a gene expressed in the caudal hindbrain during otic induction and regionalisation...
  24. Whitfield T. Zebrafish as a model for hearing and deafness. J Neurobiol. 2002;53:157-71 pubmed
    ..Vestibular and auditory defects have been detected in adult animals, making the zebrafish a useful system in which to tackle the genetic causes of late onset deafness and vestibular disease. ..
  25. Wiellette E, Sive H. Early requirement for fgf8 function during hindbrain pattern formation in zebrafish. Dev Dyn. 2004;229:393-9 pubmed
    ..These data demonstrate an early, nonredundant requirement for fgf8 function in hindbrain patterning. ..
  26. Barresi M, Burton S, Dipietrantonio K, Amsterdam A, Hopkins N, Karlstrom R. Essential genes for astroglial development and axon pathfinding during zebrafish embryogenesis. Dev Dyn. 2010;239:2603-18 pubmed publisher
    ..The genes disrupted in these viral lines have all been identified, providing a powerful new resource for the study of axon guidance, glio- and neurogenesis, and neuron-glial interactions during development of the vertebrate CNS. ..
  27. Liu J, Stainier D. Tbx5 and Bmp signaling are essential for proepicardium specification in zebrafish. Circ Res. 2010;106:1818-28 pubmed publisher
    ..Altogether, these data lead us to propose that Tbx5a confers anterior lateral plate mesodermal cells the competence to respond to Bmp signals and initiate PE development. ..
  28. Song J, Kim H, Gong Z, Liu N, Lin S. Vhnf1 acts downstream of Bmp, Fgf, and RA signals to regulate endocrine beta cell development in zebrafish. Dev Biol. 2007;303:561-75 pubmed
    ..However, the integration of these signaling pathways in vivo is not fully understood. Variant hnf1 (Vhnf1) is a transcription factor involved in pancreas, liver, and kidney development and its mutation in zebrafish ..
  29. Thisse B, Wright C, Thisse C. Activin- and Nodal-related factors control antero-posterior patterning of the zebrafish embryo. Nature. 2000;403:425-8 pubmed
    ..We propose that modulation of intercellular signalling by Antivin/Activin and Nodal-related factors provides a mechanism for the graded establishment of cell fates along the antero-posterior axis of the zebrafish embryo. ..
  30. Gong H, Lin C, Chen M, Hu M, Lin G, Zhou Y, et al. Two distinct teleost hepatocyte nuclear factor 1 genes, hnf1alpha/tcf1 and hnf1beta/tcf2, abundantly expressed in liver, pancreas, gut and kidney of zebrafish. Gene. 2004;338:35-46 pubmed
    ..of zebrafish hepatocyte nuclear factor 1 (hnf1) were identified and referred to as hnf1alpha/tcf1 and hnf1beta/tcf2. Both hnf1 genes were shown to be expressed abundantly in liver, pancreas, gut and kidney...
  31. Hostetter C, Sullivan Brown J, Burdine R. Zebrafish pronephros: a model for understanding cystic kidney disease. Dev Dyn. 2003;228:514-22 pubmed
    ..In this review, we focus on the zebrafish as a model for understanding cystic kidney disease and the links between cystic kidney disease and left-right patterning. ..
  32. Matthews R, Lorent K, Russo P, Pack M. The zebrafish onecut gene hnf-6 functions in an evolutionarily conserved genetic pathway that regulates vertebrate biliary development. Dev Biol. 2004;274:245-59 pubmed
    ..Knockdown of zebrafish hnf-6 alters expression of vhnf1 and the zebrafish orthologs of other mammalian genes regulated by hnf-6...