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Gene Review

Hnf1a  -  HNF1 homeobox A

Mus musculus

Synonyms: AI323641, HNF-1-alpha, HNF-1A, HNF1, HNF1-alpha, ...
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Disease relevance of Tcf1


High impact information on Tcf1


Chemical compound and disease context of Tcf1


Biological context of Tcf1


Anatomical context of Tcf1


Associations of Tcf1 with chemical compounds


Physical interactions of Tcf1


Regulatory relationships of Tcf1


Other interactions of Tcf1

  • The extent of heterodimerization may be regulated in a tissue-specific way because freely exchangeable heterodimers are formed in Jurkat T cells transfected with HNF-1 alpha and HNF-1 beta, whereas in liver cells stable homodimers are present [10].
  • A 516-bp fragment, encompassing a DNase I-hypersensitive site associated with P2 activity that is still retained in adult liver, contains functional HNF1 and HNF6 binding sites and confers full promoter activity in transient transfections [31].
  • Transient transfection experiments confirmed that HNF1alpha directly transactivates the Npt1 promoter and that the HSS3 region contributes to this activation [4].
  • In liver plus nine extrahepatic tissues of untreated newborn 14CoS/14CoS mutant and ch/ch wild-type mice, we compared NMO1, UGT1A6, AHD3 and HNF-1 alpha mRNA levels [32].
  • Quantitation shows that the relative ratio of HNF-1 alpha to HNF-1 beta mRNAs appears lower in the colon, where AFP is not expressed [27].

Analytical, diagnostic and therapeutic context of Tcf1


  1. Hepatocyte nuclear factor-1alpha is an essential regulator of bile acid and plasma cholesterol metabolism. Shih, D.Q., Bussen, M., Sehayek, E., Ananthanarayanan, M., Shneider, B.L., Suchy, F.J., Shefer, S., Bollileni, J.S., Gonzalez, F.J., Breslow, J.L., Stoffel, M. Nat. Genet. (2001) [Pubmed]
  2. Canonical Wnt signaling in osteoblasts is required for osteoclast differentiation. Glass, D.A., Karsenty, G. Ann. N. Y. Acad. Sci. (2006) [Pubmed]
  3. Laron dwarfism and non-insulin-dependent diabetes mellitus in the Hnf-1alpha knockout mouse. Lee, Y.H., Sauer, B., Gonzalez, F.J. Mol. Cell. Biol. (1998) [Pubmed]
  4. Hepatocyte nuclear factor 1 alpha controls renal expression of the Npt1-Npt4 anionic transporter locus. Cheret, C., Doyen, A., Yaniv, M., Pontoglio, M. J. Mol. Biol. (2002) [Pubmed]
  5. Hepatocyte nuclear factor 4alpha controls the development of a hepatic epithelium and liver morphogenesis. Parviz, F., Matullo, C., Garrison, W.D., Savatski, L., Adamson, J.W., Ning, G., Kaestner, K.H., Rossi, J.M., Zaret, K.S., Duncan, S.A. Nat. Genet. (2003) [Pubmed]
  6. Hepatocyte nuclear factor 1 inactivation results in hepatic dysfunction, phenylketonuria, and renal Fanconi syndrome. Pontoglio, M., Barra, J., Hadchouel, M., Doyen, A., Kress, C., Bach, J.P., Babinet, C., Yaniv, M. Cell (1996) [Pubmed]
  7. Transcription factor NF-E2 is required for platelet formation independent of the actions of thrombopoietin/MGDF in megakaryocyte development. Shivdasani, R.A., Rosenblatt, M.F., Zucker-Franklin, D., Jackson, C.W., Hunt, P., Saris, C.J., Orkin, S.H. Cell (1995) [Pubmed]
  8. A common regulatory locus affects both HNF4/HNF1alpha pathway activation and sensitivity to LPS-mediated apoptosis in rat hepatoma cells. Bulla, G.A., Givens, E., Brown, S., Oladiran, B., Kraus, D. J. Cell. Sci. (2001) [Pubmed]
  9. Redundant regulation of T cell differentiation and TCRalpha gene expression by the transcription factors LEF-1 and TCF-1. Okamura, R.M., Sigvardsson, M., Galceran, J., Verbeek, S., Clevers, H., Grosschedl, R. Immunity (1998) [Pubmed]
  10. HNF-1 alpha and HNF-1 beta (vHNF-1) share dimerization and homeo domains, but not activation domains, and form heterodimers in vitro. Mendel, D.B., Hansen, L.P., Graves, M.K., Conley, P.B., Crabtree, G.R. Genes Dev. (1991) [Pubmed]
  11. Hepatocyte nuclear factor-1 acts as an accessory factor to enhance the inhibitory action of insulin on mouse glucose-6-phosphatase gene transcription. Streeper, R.S., Eaton, E.M., Ebert, D.H., Chapman, S.C., Svitek, C.A., O'Brien, R.M. Proc. Natl. Acad. Sci. U.S.A. (1998) [Pubmed]
  12. The transcriptional activator hepatocyte nuclear factor 6 regulates liver gene expression. Samadani, U., Costa, R.H. Mol. Cell. Biol. (1996) [Pubmed]
  13. Lef1 is required for the transition of Wnt signaling from mesenchymal to epithelial cells in the mouse embryonic mammary gland. Boras-Granic, K., Chang, H., Grosschedl, R., Hamel, P.A. Dev. Biol. (2006) [Pubmed]
  14. HNF1alpha is involved in tissue-specific regulation of CFTR gene expression. Mouchel, N., Henstra, S.A., McCarthy, V.A., Williams, S.H., Phylactides, M., Harris, A. Biochem. J. (2004) [Pubmed]
  15. The transcription factor hepatocyte nuclear factor-6 controls the development of pancreatic ducts in the mouse. Pierreux, C.E., Poll, A.V., Kemp, C.R., Clotman, F., Maestro, M.A., Cordi, S., Ferrer, J., Leyns, L., Rousseau, G.G., Lemaigre, F.P. Gastroenterology (2006) [Pubmed]
  16. Involvement of hepatocyte nuclear factor 3 in endoderm differentiation of embryonic stem cells. Levinson-Dushnik, M., Benvenisty, N. Mol. Cell. Biol. (1997) [Pubmed]
  17. Cloning of cDNA and the gene encoding human hepatocyte nuclear factor (HNF)-3 beta and mutation screening in Japanese subjects with maturity-onset diabetes of the young. Yamada, S., Zhu, Q., Aihara, Y., Onda, H., Zhang, Z., Yu, L., Jin, L., Si, Y.J., Nishigori, H., Tomura, H., Inoue, I., Morikawa, A., Yamagata, K., Hanafusa, T., Matsuzawa, Y., Takeda, J. Diabetologia (2000) [Pubmed]
  18. Bile acids regulate gluconeogenic gene expression via small heterodimer partner-mediated repression of hepatocyte nuclear factor 4 and Foxo1. Yamagata, K., Daitoku, H., Shimamoto, Y., Matsuzaki, H., Hirota, K., Ishida, J., Fukamizu, A. J. Biol. Chem. (2004) [Pubmed]
  19. A transcriptional hierarchy involved in mammalian cell-type specification. Kuo, C.J., Conley, P.B., Chen, L., Sladek, F.M., Darnell, J.E., Crabtree, G.R. Nature (1992) [Pubmed]
  20. In murine 3T3 fibroblasts, different second messenger pathways resulting in the induction of NO synthase II (iNOS) converge in the activation of transcription factor NF-kappaB. Kleinert, H., Euchenhofer, C., Ihrig-Biedert, I., Förstermann, U. J. Biol. Chem. (1996) [Pubmed]
  21. Regulated expression of human angiotensinogen gene by hepatocyte nuclear factor 4 and chicken ovalbumin upstream promoter-transcription factor. Yanai, K., Hirota, K., Taniguchi-Yanai, K., Shigematsu, Y., Shimamoto, Y., Saito, T., Chowdhury, S., Takiguchi, M., Arakawa, M., Nibu, Y., Sugiyama, F., Yagami, K., Fukamizu, A. J. Biol. Chem. (1999) [Pubmed]
  22. Tissue-specific regulation of mouse hepatocyte nuclear factor 4 expression. Zhong, W., Mirkovitch, J., Darnell, J.E. Mol. Cell. Biol. (1994) [Pubmed]
  23. Transcription factor complex formation and chromatin fine structure alterations at the murine c-fms (CSF-1 receptor) locus during maturation of myeloid precursor cells. Tagoh, H., Himes, R., Clarke, D., Leenen, P.J., Riggs, A.D., Hume, D., Bonifer, C. Genes Dev. (2002) [Pubmed]
  24. Oxidative stress activates metal-responsive transcription factor-1 binding activity. Occupancy in vivo of metal response elements in the metallothionein-I gene promoter. Dalton, T.P., Li, Q., Bittel, D., Liang, L., Andrews, G.K. J. Biol. Chem. (1996) [Pubmed]
  25. Molecular cloning of mouse connexins26 and -32: similar genomic organization but distinct promoter sequences of two gap junction genes. Hennemann, H., Kozjek, G., Dahl, E., Nicholson, B., Willecke, K. Eur. J. Cell Biol. (1992) [Pubmed]
  26. Functional dissection of the brain-specific rat aldolase C gene promoter in transgenic mice. Essential role of two GC-rich boxes and an HNF3 binding site. Thomas, M., Skala, H., Kahn, A., Tuy, F.P. J. Biol. Chem. (1995) [Pubmed]
  27. HNF-1 alpha and HNF-1 beta expression in mouse intestinal crypts. Serfas, M.S., Tyner, A.L. Am. J. Physiol. (1993) [Pubmed]
  28. Constitutive expression of murine complement factor B gene is regulated by the interaction of its upstream promoter with hepatocyte nuclear factor 4. Garnier, G., Circolo, A., Colten, H.R. J. Biol. Chem. (1996) [Pubmed]
  29. Role of 15-deoxy delta(12,14) prostaglandin J2 and Nrf2 pathways in protection against acute lung injury. Mochizuki, M., Ishii, Y., Itoh, K., Iizuka, T., Morishima, Y., Kimura, T., Kiwamoto, T., Matsuno, Y., Hegab, A.E., Nomura, A., Sakamoto, T., Uchida, K., Yamamoto, M., Sekizawa, K. Am. J. Respir. Crit. Care Med. (2005) [Pubmed]
  30. Transcription factor decoy for NFkappaB inhibits TNF-alpha-induced cytokine and adhesion molecule expression in vivo. Tomita, N., Morishita, R., Tomita, S., Gibbons, G.H., Zhang, L., Horiuchi, M., Kaneda, Y., Higaki, J., Ogihara, T., Dzau, V.J. Gene Ther. (2000) [Pubmed]
  31. Expression of the alpha7 isoform of hepatocyte nuclear factor (HNF) 4 is activated by HNF6/OC-2 and HNF1 and repressed by HNF4alpha1 in the liver. Briançon, N., Bailly, A., Clotman, F., Jacquemin, P., Lemaigre, F.P., Weiss, M.C. J. Biol. Chem. (2004) [Pubmed]
  32. Extrahepatic expression of NAD(P)H:menadione oxidoreductase, UDP glucuronosyltransferase-1A6, microsomal aldehyde dehydrogenase, and hepatic nuclear factor-1 alpha mRNAs in ch/ch and 14CoS/14CoS mice. Vasiliou, V., Reuter, S.F., Nebert, D.W. Biochem. Biophys. Res. Commun. (1997) [Pubmed]
  33. Expression of transcription factor HNF-4 in the extraembryonic endoderm, gut, and nephrogenic tissue of the developing mouse embryo: HNF-4 is a marker for primary endoderm in the implanting blastocyst. Duncan, S.A., Manova, K., Chen, W.S., Hoodless, P., Weinstein, D.C., Bachvarova, R.F., Darnell, J.E. Proc. Natl. Acad. Sci. U.S.A. (1994) [Pubmed]
  34. Molecular cloning, functional expression, and chromosomal localization of mouse hepatocyte nuclear factor 1. Kuo, C.J., Conley, P.B., Hsieh, C.L., Francke, U., Crabtree, G.R. Proc. Natl. Acad. Sci. U.S.A. (1990) [Pubmed]
  35. Quantitative gene expression profiling reveals a fetal hepatic phenotype of murine ES-derived hepatocytes. Jochheim, A., Hillemann, T., Kania, G., Scharf, J., Attaran, M., Manns, M.P., Wobus, A.M., Ott, M. Int. J. Dev. Biol. (2004) [Pubmed]
  36. Liver-specific reactivation of the inactivated Hnf-1alpha gene: elimination of liver dysfunction to establish a mouse MODY3 model. Lee, Y.H., Magnuson, M.A., Muppala, V., Chen, S.S. Mol. Cell. Biol. (2003) [Pubmed]
  37. Hepatic nuclear factor 1 (HNF1) shows a wider distribution than products of its known target genes in developing mouse. Blumenfeld, M., Maury, M., Chouard, T., Yaniv, M., Condamine, H. Development (1991) [Pubmed]
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