Abstract
Maturity-onset diabetes of the young (MODY) is a heterogeneous sub-type of diabetes mellitus that was originally defined as “fasting hyperglycaemia diagnosed before age 25 which could be treated without insulin for more than 2 years” (Tattersall & Fajans, 1975). In addition to the early age of onset, the main distinguishing feature relative to the common forms of non-insulin-dependent diabetes mellitus is that it is characterized by autosomal dominant inheritance (Hattersley, 1998; Tattersall, 1998). Mutations in five genes have been shown to cause it in addition to as yet unidentified forms of the disease (Chevre et al., 1998). The first of these that was identified was the glucokinase gene (MODY-2), followed by hepatocyte nuclear factor 4α and hepatocyte nuclear factor 1α, and more recently the genes for insulin-promoter factor 1 and hepatocyte nuclear factor 1β.
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Agius, L., Aiston, S., Newgard, C.B. (2000). The Control Strength of Glucokinase in Hepatocytes: A Predictor of Metabolic Defects in Maturity Onset Diabetes of the Young, Type 2 . In: Cornish-Bowden, A., Cárdenas, M.L. (eds) Technological and Medical Implications of Metabolic Control Analysis. NATO Science Series, vol 74. Springer, Dordrecht. https://doi.org/10.1007/978-94-011-4072-0_11
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DOI: https://doi.org/10.1007/978-94-011-4072-0_11
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