The Control Strength of Glucokinase in hepatocytes: A Predictor of Metabolic Defects in Maturity Onset Diabetes of the Young, Type 2

摘要

Maturity-onset diabetes of the young (MODY) is a heterogeneous sub-type of diabetes mellitus that was originally defined as "fasting hyperglycaemia diagnsed 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 distingushing 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 havee 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 4 alpha and hepatocyte nuclear factor 1 alpha , and more recently the genes for insulin-promoter factor 1 and hepatocyte nuclear factor 1 beta .