Contrasting Diabetes Phenotypes Associated With Hepatocyte Nuclear Factor-1 and -1 Mutations
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منابع مشابه
Contrasting Diabetes Phenotypes Associated With Hepatocyte Nuclear Factor-1 and -1 Mutations
OBJECTIVE — Mutations in the highly homologous transcription factors hepatocyte nuclear factor (HNF)-1 and -1 cause maturity-onset diabetes of the young types 3 and 5, respectively. Diabetes due to HNF-1 mutations is well characterized. However, physiological assessment of the HNF-1 phenotype is limited. We aimed to test the hypothesis that the diabetes phenotype due to HNF-1 mutations is simil...
متن کاملContrasting Insulin Sensitivity of Endogenous Glucose Production Rate in Subjects With Hepatocyte Nuclear Factor-1 and -1 Mutations
Heterozygous mutations in the transcription factors hepatocyte nuclear factor (HNF)-1 and -1 result in MODY (maturity-onset diabetes of the young). Despite structural similarity between HNF-1 and -1 , HNF-1 mutation carriers have hyperinsulinemia, whereas HNF-1 mutation carriers have normal or reduced insulin concentrations. We examined whether HNF-1 mutation carriers are insulin resistant. The...
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Hypertension in spontaneously hypertensive rat (SHR) is associated with renal redox stress, and we hypothesized that nephropathy arises in SHR-A3 from altered capacity to mitigate redox stress compared with nephropathy-resistant SHR lines. We measured renal expression of redox genes in distinct lines of the spontaneously hypertensive rat (SHR-A3, SHR-B2, SHR-C) and the normotensive Wistar-Kyoto...
متن کامل-Cell Dysfunction, Insulin Sensitivity, and Glycosuria Precede Diabetes in Hepatocyte Nuclear Factor-1 Mutation Carriers
RESULTS — HNF-1 mutations were found in 20 offspring, 7 with diabetes and 13 without diabetes. The 13 nondiabetic mutation carriers were compared with 27 family control subjects, who were matched for age, sex, and BMI. There was marked -cell deficiency with reduced insulinogenic index (53.5 [31.5–90.9] vs. 226.0 [126.0–407.1], SD [range], P 0.001) and area under the curve for insulin (P 0.001)....
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The hepatocyte nuclear factor-1beta encoded by the TCF2 gene plays a role for the specific regulation of gene expression in various tissues such as liver, kidney, intestine, and pancreatic islets and is involved in the embryonic development of these organs. TCF2 mutations are known to be responsible for the maturity-onset diabetes of the young type 5 associated with renal manifestations. Severa...
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ژورنال
عنوان ژورنال: Diabetes Care
سال: 2004
ISSN: 0149-5992,1935-5548
DOI: 10.2337/diacare.27.5.1102