Liver-Specific Reactivation of the Inactivated Hnf-1α Gene: Elimination of Liver Dysfunction To Establish a Mouse MODY3 Model

REFERENCES

• Baumhueter, S., D. B. Mendel, P. B. Conley, C. J. Kuo, C. Turk, M. K. Graves, C. A. Edwards, G. Courtois, and G. R. Crabtree. 1990. HNF-1 shares three sequence motifs with the POU domain proteins and is identical to LF-B1 and APF. Genes Dev. 4: 372–379.
   PubMedGoogle Scholar
• Bell, G. I., and K. S. Polonsky. 2001. Diabetes mellitus and genetically programmed defects in β-cell function. Nature 414: 788–791.
   PubMed Web of Science ®Google Scholar
• Blumenfeld, M., M. Maury, T. Chouard, M. Yaniv, and H. Condamine. 1991. Hepatic nuclear factor 1 (HNF 1) shows a wider distribution than products of its known target genes in developing mouse. Development 113: 589–599.
   PubMedGoogle Scholar
• Carroll, N. V., R. W. Longley, and J. H. Roe. 1955. The determination of glycogen in liver and muscle by use of anthrone reagent. J. Biol. Chem. 212: 335–343.
   PubMedGoogle Scholar
• De Simone, W., L. De Magistris, D. Lazzaro, J. Gerstner, P. Monaci, A. Nicosia and R. Cortese. 1991. LFB3, a heterodimer-forming homeoprotein of the LEB1 family, is expressed in specialized epithelia. EMBO J. 10: 1435–1443.
   PubMed Web of Science ®Google Scholar
• Dukes, I. D., S. Sreenan, M. W. Roe, M. Levisetti, Y. P. Zhou, D. Ostrega, G. I. Bell, M. Pontoglio, M. Yaniv, L. Philipson, and K. S. Polonsky. 1998. Defective pancreatic-cell glycolytic signaling in hepatocyte nuclear factor-1-deficient mice. J. Biol. Chem. 273: 24457–24464.
   PubMed Web of Science ®Google Scholar
• Duncan, S. A., A. Nagy and W. Chan. 1997. Murine gastrulation requires HNF-4 regulated gene expression in the visceral endoderm: tetraploid rescue of Hnf-4(-/-) embryos. Development 124: 279–287.
   PubMedGoogle Scholar
• Fajans, S. S., G. I. Bell, and K. S. Polonsky. 2001. Molecular mechanisms and clinical pathophysiology of maturity-onset diabetes of the young. N. Engl. J. Med. 345: 971–980.
   PubMed Web of Science ®Google Scholar
• Habener, J. F., and D. A. Stoffers. 1998. A newly discovered role of transcription factors involved in pancreas development and the pathogenesis of diabetes mellitus. Proc. Assoc. Am. Physicians 110: 12–21.
   PubMedGoogle Scholar
• Hedemann, M. S., A. R. Pedersen, and R. M. Engberg. 2001. Exocrine pancreatic secretion is stimulated in piglets fed fish oil compared with those fed coconut oil or lard. J. Nutr. 131: 3222–3226.
   PubMed Web of Science ®Google Scholar
• Hiraiwa, H., C.-J. Pan, B. Lin, T. E. Akiyama, F. J. Gonzalez, and J. Y. Chou. 2001. A molecular link between the common phenotypes of type 1 glycogen storage disease and HNF1α-null mice. J. Biol. Chem. 276: 7963–7967.
   PubMed Web of Science ®Google Scholar
• Hogan, B., R. Bedington, F. Costantini, and E. Lacy. 1994. Manipulating the mouse embryo: a laboratory manual, 2nd ed. Cold Spring Harbor Laboratory Press, Cold Spring Harbor, N.Y.
   Google Scholar
• Howard, B. V. 1985. Lipoprotein metabolism in diabetes mellitus. J. Lipid Res. 28: 613–628.
   Google Scholar
• Lawrence, J. C., Jr., and P. J. Roach. 1997. New insights into the role and mechanism of glycogen synthase activation by insulin. Diabetes 46: 541–547.
   PubMed Web of Science ®Google Scholar
• Lee, Y. H., B. Sauer, and F. J. Gonzalez. 1998. Laron dwarfism and non-insulin-dependent diabetes mellitus in the Hnf-1α Knockout mice. Mol. Cell. Biol. 18: 3059–3068.
   PubMed Web of Science ®Google Scholar
• Liu, M., S. J. Pleasure, A. E. Collins, J. L. Noebels, F. J. Naya, M. J. Tsai, and D. H. Lowenstein. 2000. Loss of BETA2/NeuroD leads to malformation of the dentate gyrus and epilepsy. Proc. Natl. Acad. Sci. USA 97: 865–870.
   PubMedGoogle Scholar
• Mandal, A. K., Z. Zhang, J. Y. Chou, and A. B. Mukherjee. 2001. Pancreatic phospholipase A2 via its receptor regulates expression of key enzymes of phospholipid and sphingolipid metabolism. FASEB J. 15: 1834–1836.
   PubMedGoogle Scholar
• Muppala, V., C.-S. Lin, and Y. H. Lee. 2000. The role of HNF-1α in controlling hepatic catalase activity. Mol. Pharmacol. 57: 93–100.
   PubMedGoogle Scholar
• O'Looney, P., D. Irwin, P. Briscoe, and G. V. Vahouny. 1985. Lipoprotein composition as a component in the lipoprotein clearance defect in experimental diabetes. J. Biol. Chem. 260: 428–432.
   PubMedGoogle Scholar
• Pontoglio, M., J. Barra, M. Hadchouel, A. Doen, C. Dress, J. P. Bach, C. Babinet, and M. Yaniv. 1996. Hepatocyte nuclear factor 1 inactivation results in hepatic dysfunction, phenylketonuria, and renal Fanconi syndrome. Cell 84: 575–585.
   PubMed Web of Science ®Google Scholar
• Pontoglio, M., B. Sreenan, M. Roe, W. Pugh, D. Ostrega, A. Doyen, A. J. Pick, A. Baldwin, G. Velho, P. Froguel, M. Levisetti, S. Bonner-Weir, G. I. Bell, M. Yaniv, and K. S. Polonsky. 1998. Defective insulin secretion in hepatocyte nuclear factor 1α-deficient mice. J. Clin. Investig. 101: 2215–2222.
   PubMed Web of Science ®Google Scholar
• Postic, C., M. Shiota, K. D. Niswender, T. L. Jetton, Y. Chen, J. M. Moates, K. D. Shelton, J. Lindner, A. D. Cherrington, and M. A. Magnuson. 1999. Dual roles for glucokinase in glucose homeostasis as determined by liver and pancreatic β cell-specific gene knock-outs using cre recombinase. J. Biol. Chem. 274: 305–315.
   PubMed Web of Science ®Google Scholar
• Postic, C., M. Shiota, and M. A. Magnuson. 2001. Cell-specific roles of glucokinase in glucose homeostasis. Recent Prog. Horm. Res. 56: 195–217.
   PubMedGoogle Scholar
• Shih, D. Q., M. Bussen, E. Sehayek, M. Ananthanarayanan, B. L. Shneider, F. J. Suchy, S. Shefer, J. S. Bollileni, F. J. Gonzalez, J. L. Breslow and M. Stoffel. 2001. Hepatocyte nuclear factor-1α is an essential regulator of bile acid and plasma cholesterol metabolism. Nat. Genet. 27: 375–382.
   PubMed Web of Science ®Google Scholar
• Shih, D. Q., S. Screenan, K. N. Nunoz, L. Philipson, M. Pontogolio, M. Yaniv, K. S. Polonsky, and M. Stoffel. 2001. Loss of HNF-1α function in mice leads to abnormal expression of genes involved in pancreatic islet development and metabolism. Diabetes 50: 2472–2480.
   PubMed Web of Science ®Google Scholar
• Stephanova, E., F. Tissir, N. Dusetti, J. Iovanna, and C. Szpirer. 1996. The rat genes encoding the pancreatitis-associated proteins I, II, and III (Pap1, Pap2, Pap3) and the lithostatin/pancreatic stone protein/regeneration protein (reg) colocalize at 4q33-4q34. Cytogenet. Cell Genet. 72: 83–85.
   PubMedGoogle Scholar
• van Steenbergen, W., and S. Lanckmans. 1995. Liver disturbances in obesity and diabetes mellitus. Int. J. Obes. Relat. Metab. Disord. 19(Suppl. 3): S27–S36.
   PubMedGoogle Scholar
• Yamagata, K., N. Oda, P. J. Kaisaki, S. Menzel, H. Furuta, M. Vaxillaire, L. Southam, R. D. Cox, G. M. Lathrop, V. V. Borirai, X. Chen, N. J. Cox, Y. Oda, H. Yano, M. M. Le Beau, S. Yamad, H. Nishigori, J. Takeda, S. S. Fajans, A. T. Hattersley, N. Iwasaki, T. Hansen, O. Pedersen, K. S. Polonsky, R. C. Turner, G. Velho, J.-C. Chevre, P. Froguel, and G. I. Bell. 1996. Mutations in the hepatocyte nuclear factor-1α gene in maturity-onset diabetes of the young (MODY3). Nature 384: 455–458.
   PubMed Web of Science ®Google Scholar
• Zenilman, M. E., T. H. Magnuson, K. Swinson, J. Egan, R. Perfetti, A. R. Shuldiner. 1996. Pancreatic thread protein in mitogenic to pancreatic-derived cells in culture. Gastroenterology 110: 1208–1214.
   PubMed Web of Science ®Google Scholar
• Zenilman, M. E., D. Tuchman, W. Zheng, J. Levine, and H. Delany. 2000. Comparison of reg I and reg III levels during acute pancreatitis in the rat. Ann. Surg. 232: 646–652.
   PubMedGoogle Scholar