PubMed

Experimental models of transcription factor-associated maturity-onset diabetes of the young.

Wang H, Hagenfeldt-Johansson K, Otten LA, Gauthier BR, Herrera PL, Wollheim CB.

Diabetes. 2002 Dec;51 Suppl 3:S333-42. Review.PMID: 12475772 [PubMed - indexed for MEDLINE]Related articlesFree article

Hepatocyte nuclear factor-1alpha modulates pancreatic beta-cell growth by regulating the expression of insulin-like growth factor-1 in INS-1 cells.

Yang Q, Yamagata K, Fukui K, Cao Y, Nammo T, Iwahashi H, Wang H, Matsumura I, Hanafusa T, Bucala R, Wollheim CB, Miyagawa J, Matsuzawa Y.

Diabetes. 2002 Jun;51(6):1785-92.PMID: 12031966 [PubMed - indexed for MEDLINE]Related articlesFree article

Dominant-negative suppression of HNF-1alpha function results in defective insulin gene transcription and impaired metabolism-secretion coupling in a pancreatic beta-cell line.

Wang H, Maechler P, Hagenfeldt KA, Wollheim CB.

EMBO J. 1998 Nov 16;17(22):6701-13.PMID: 9822613 [PubMed - indexed for MEDLINE]Related articlesFree article

Mutation screening in 18 Caucasian families suggest the existence of other MODY genes.

Chèvre JC, Hani EH, Boutin P, Vaxillaire M, Blanché H, Vionnet N, Pardini VC, Timsit J, Larger E, Charpentier G, Beckers D, Maes M, Bellanné-Chantelot C, Velho G, Froguel P.

Diabetologia. 1998 Sep;41(9):1017-23.PMID: 9754819 [PubMed - indexed for MEDLINE]Related articles

Profound defects in pancreatic beta-cell function in mice with combined heterozygous mutations in Pdx-1, Hnf-1alpha, and Hnf-3beta.

Shih DQ, Heimesaat M, Kuwajima S, Stein R, Wright CV, Stoffel M.

Proc Natl Acad Sci U S A. 2002 Mar 19;99(6):3818-23.PMID: 11904435 [PubMed - indexed for MEDLINE]Related articlesFree article

Hepatocyte nuclear factor 1 alpha (HNF-1 alpha) mutations in maturity-onset diabetes of the young.

Ellard S.

Hum Mutat. 2000 Nov;16(5):377-85. Review.PMID: 11058894 [PubMed - indexed for MEDLINE]Related articles

Mutations in the hepatocyte nuclear factor-4alpha gene in maturity-onset diabetes of the young (MODY1)

Yamagata K, Furuta H, Oda N, Kaisaki PJ, Menzel S, Cox NJ, Fajans SS, Signorini S, Stoffel M, Bell GI.

Nature. 1996 Dec 5;384(6608):458-60.PMID: 8945471 [PubMed - indexed for MEDLINE]Related articles

Genetic and clinical characterisation of maturity-onset diabetes of the young in Spanish families.

Costa A, Bescós M, Velho G, Chêvre J, Vidal J, Sesmilo G, Bellanné-Chantelot C, Froguel P, Casamitjana R, Rivera-Fillat F, Gomis R, Conget I.

Eur J Endocrinol. 2000 Apr;142(4):380-6.PMID: 10754480 [PubMed - indexed for MEDLINE]Related articlesFree article

Beta-cell-targeted expression of a dominant-negative hepatocyte nuclear factor-1 alpha induces a maturity-onset diabetes of the young (MODY)3-like phenotype in transgenic mice.

Hagenfeldt-Johansson KA, Herrera PL, Wang H, Gjinovci A, Ishihara H, Wollheim CB.

Endocrinology. 2001 Dec;142(12):5311-20.PMID: 11713231 [PubMed - indexed for MEDLINE]Related articlesFree article

Human insulin gene is a target gene of hepatocyte nuclear factor-1alpha (HNF-1alpha) and HNF-1beta.

Okita K, Yang Q, Yamagata K, Hangenfeldt KA, Miyagawa J, Kajimoto Y, Nakajima H, Namba M, Wollheim CB, Hanafusa T, Matsuzawa Y.

Biochem Biophys Res Commun. 1999 Sep 24;263(2):566-9.PMID: 10491332 [PubMed - indexed for MEDLINE]Related articles

[Molecular background and clinical characteristics of autosomal dominant type 2 diabetes mellitus]

Małecki M, Krolewski AS.

Przegl Lek. 2000;57 Suppl 3:13-8. Review. Polish. PMID: 11293229 [PubMed - indexed for MEDLINE]Related articles

Defective insulin secretion in hepatocyte nuclear factor 1alpha-deficient mice.

Pontoglio M, Sreenan S, Roe M, Pugh W, Ostrega D, Doyen A, Pick AJ, Baldwin A, Velho G, Froguel P, Levisetti M, Bonner-Weir S, Bell GI, Yaniv M, Polonsky KS.

J Clin Invest. 1998 May 15;101(10):2215-22.PMID: 9593777 [PubMed - indexed for MEDLINE]Related articlesFree article

A distant upstream promoter of the HNF-4alpha gene connects the transcription factors involved in maturity-onset diabetes of the young.

Thomas H, Jaschkowitz K, Bulman M, Frayling TM, Mitchell SM, Roosen S, Lingott-Frieg A, Tack CJ, Ellard S, Ryffel GU, Hattersley AT.

Hum Mol Genet. 2001 Sep 15;10(19):2089-97.PMID: 11590126 [PubMed - indexed for MEDLINE]Related articlesFree article

Regulation of apolipoprotein M gene expression by MODY3 gene hepatocyte nuclear factor-1alpha: haploinsufficiency is associated with reduced serum apolipoprotein M levels.

Richter S, Shih DQ, Pearson ER, Wolfrum C, Fajans SS, Hattersley AT, Stoffel M.

Diabetes. 2003 Dec;52(12):2989-95.PMID: 14633861 [PubMed - indexed for MEDLINE]Related articlesFree article

beta-cell genes and diabetes: quantitative and qualitative differences in the pathophysiology of hepatic nuclear factor-1alpha and glucokinase mutations.

Pearson ER, Velho G, Clark P, Stride A, Shepherd M, Frayling TM, Bulman MP, Ellard S, Froguel P, Hattersley AT.

Diabetes. 2001 Feb;50 Suppl 1:S101-7.PMID: 11272165 [PubMed - indexed for MEDLINE]Related articles

Mutations in the genes for hepatocyte nuclear factor (HNF)-1alpha, -4alpha, -1beta, and -3beta; the dimerization cofactor of HNF-1; and insulin promoter factor 1 are not common causes of early-onset type 2 diabetes in Pima Indians.

Baier LJ, Permana PA, Traurig M, Dobberfuhl A, Wiedrich C, Sutherland J, Thuillez P, Luczy-Bachman G, Hara M, Horikawa Y, Hinokio Y, Hanson RL, Bogardus C.

Diabetes Care. 2000 Mar;23(3):302-4.PMID: 10868855 [PubMed - indexed for MEDLINE]Related articlesFree article

Functional study of the E276Q mutant hepatocyte nuclear factor-4alpha found in type 1 maturity-onset diabetes of the young: impaired synergy with chicken ovalbumin upstream promoter transcription factor II on the hepatocyte nuclear factor-1 promoter.

Suaud L, Hemimou Y, Formstecher P, Laine B.

Diabetes. 1999 May;48(5):1162-7.PMID: 10331424 [PubMed - indexed for MEDLINE]Related articlesFree article

Hepatic function in a family with a nonsense mutation (R154X) in the hepatocyte nuclear factor-4alpha/MODY1 gene.

Lindner T, Gragnoli C, Furuta H, Cockburn BN, Petzold C, Rietzsch H, Weiss U, Schulze J, Bell GI.

J Clin Invest. 1997 Sep 15;100(6):1400-5.PMID: 9294105 [PubMed - indexed for MEDLINE]Related articlesFree article

beta-cell genes and diabetes: molecular and clinical characterization of mutations in transcription factors.

Frayling TM, Evans JC, Bulman MP, Pearson E, Allen L, Owen K, Bingham C, Hannemann M, Shepherd M, Ellard S, Hattersley AT.

Diabetes. 2001 Feb;50 Suppl 1:S94-100.PMID: 11272211 [PubMed - indexed for MEDLINE]Related articles

Decreased glibenclamide uptake in hepatocytes of hepatocyte nuclear factor-1alpha-deficient mice: a mechanism for hypersensitivity to sulfonylurea therapy in patients with maturity-onset diabetes of the young, type 3 (MODY3).

Boileau P, Wolfrum C, Shih DQ, Yang TA, Wolkoff AW, Stoffel M.

Diabetes. 2002 Dec;51 Suppl 3:S343-8.PMID: 12475773 [PubMed - indexed for MEDLINE]Related articlesFree article