PubMed

The generalized aminoaciduria seen in patients with hepatocyte nuclear factor-1alpha mutations is a feature of all patients with diabetes and is associated with glucosuria.

Bingham C, Ellard S, Nicholls AJ, Pennock CA, Allen J, James AJ, Satchell SC, Salzmann MB, Hattersley AT.

Diabetes. 2001 Sep;50(9):2047-52.PMID: 11522670 [PubMed - indexed for MEDLINE]Related articlesFree article

A low renal threshold for glucose in diabetic patients with a mutation in the hepatocyte nuclear factor-1alpha (HNF-1alpha) gene.

Menzel R, Kaisaki PJ, Rjasanowski I, Heinke P, Kerner W, Menzel S.

Diabet Med. 1998 Oct;15(10):816-20.PMID: 9796880 [PubMed - indexed for MEDLINE]Related articles

Contrasting diabetes phenotypes associated with hepatocyte nuclear factor-1alpha and -1beta mutations.

Pearson ER, Badman MK, Lockwood CR, Clark PM, Ellard S, Bingham C, Hattersley AT.

Diabetes Care. 2004 May;27(5):1102-7.PMID: 15111528 [PubMed - indexed for MEDLINE]Related articlesFree article

Beta-cell dysfunction, insulin sensitivity, and glycosuria precede diabetes in hepatocyte nuclear factor-1alpha mutation carriers.

Stride A, Ellard S, Clark P, Shakespeare L, Salzmann M, Shepherd M, Hattersley AT.

Diabetes Care. 2005 Jul;28(7):1751-6.PMID: 15983330 [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

Variants in the hepatocyte nuclear factor-1alpha and -4alpha genes in Finnish and Chinese subjects with late-onset type 2 diabetes.

Rissanen J, Wang H, Miettinen R, Kärkkäinen P, Kekäläinen P, Mykkänen L, Kuusisto J, Karhapää P, Niskanen L, Uusitupa M, Laakso M.

Diabetes Care. 2000 Oct;23(10):1533-8.PMID: 11023148 [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 hepatocyte nuclear factor-1alpha gene in Caucasian families originally classified as having Type I diabetes.

Møller AM, Dalgaard LT, Pociot F, Nerup J, Hansen T, Pedersen O.

Diabetologia. 1998 Dec;41(12):1528-31.PMID: 9867222 [PubMed - indexed for MEDLINE]Related articles

Identification and functional analysis of mutations in the hepatocyte nuclear factor-1alpha gene in anti-islet autoantibody-negative Japanese patients with type 1 diabetes.

Kawasaki E, Sera Y, Yamakawa K, Abe T, Ozaki M, Uotani S, Ohtsu N, Takino H, Yamasaki H, Yamaguchi Y, Matsuura N, Eguchi K.

J Clin Endocrinol Metab. 2000 Jan;85(1):331-5.PMID: 10634407 [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

Three new mutations in the hepatocyte nuclear factor-1alpha gene in Japanese subjects with diabetes mellitus: clinical features and functional characterization.

Yoshiuchi I, Yamagata K, Yang Q, Iwahashi H, Okita K, Yamamoto K, Oue T, Imagawa A, Hamaguchi T, Yamasaki T, Horikawa Y, Satoh T, Nakajima H, Miyazaki J, Higashiyama S, Miyagawa J, Namba M, Hanafusa T, Matsuzawa Y.

Diabetologia. 1999 May;42(5):621-6.PMID: 10333057 [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

Serum amino acids in patients with mutations in the hepatocyte nuclear factor-1 alpha gene.

Stride A, Pearson ER, Brown A, Gooding K, Castleden HA, Hattersley AT.

Diabet Med. 2004 Aug;21(8):928-30.PMID: 15270800 [PubMed - indexed for MEDLINE]Related articles

A prevalent amino acid polymorphism at codon 98 in the hepatocyte nuclear factor-1alpha gene is associated with reduced serum C-peptide and insulin responses to an oral glucose challenge.

Urhammer SA, Fridberg M, Hansen T, Rasmussen SK, Møller AM, Clausen JO, Pedersen O.

Diabetes. 1997 May;46(5):912-6.PMID: 9133564 [PubMed - indexed for MEDLINE]Related articles

Liver and kidney function in Japanese patients with maturity-onset diabetes of the young.

Iwasaki N, Ogata M, Tomonaga O, Kuroki H, Kasahara T, Yano N, Iwamoto Y.

Diabetes Care. 1998 Dec;21(12):2144-8.PMID: 9839108 [PubMed - indexed for MEDLINE]Related articlesFree article

Sensitivity to sulphonylureas in patients with hepatocyte nuclear factor-1alpha gene mutations: evidence for pharmacogenetics in diabetes.

Pearson ER, Liddell WG, Shepherd M, Corrall RJ, Hattersley AT.

Diabet Med. 2000 Jul;17(7):543-5.PMID: 10972586 [PubMed - indexed for MEDLINE]Related articles

Clinical spectrum associated with hepatocyte nuclear factor-1beta mutations.

Bellanné-Chantelot C, Chauveau D, Gautier JF, Dubois-Laforgue D, Clauin S, Beaufils S, Wilhelm JM, Boitard C, Noël LH, Velho G, Timsit J.

Ann Intern Med. 2004 Apr 6;140(7):510-7.PMID: 15068978 [PubMed - indexed for MEDLINE]Related articlesFree article

Chronic diabetic complications in patients with MODY3 diabetes.

Isomaa B, Henricsson M, Lehto M, Forsblom C, Karanko S, Sarelin L, Häggblom M, Groop L.

Diabetologia. 1998 Apr;41(4):467-73.PMID: 9562352 [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

Molecular genetics of diabetes mellitus in Chinese subjects: identification of mutations in glucokinase and hepatocyte nuclear factor-1alpha genes in patients with early-onset type 2 diabetes mellitus/MODY.

Ng MC, Cockburn BN, Lindner TH, Yeung VT, Chow CC, So WY, Li JK, Lo YM, Lee ZS, Cockram CS, Critchley JA, Bell GI, Chan JC.

Diabet Med. 1999 Nov;16(11):956-63.PMID: 10588527 [PubMed - indexed for MEDLINE]Related articles