Brief Genetics Report: Insulin promoter Factor 1 Gene Is Not a Major Cause of Maturity-Onset Diabetes of the Young in French Caucasians

摘要

Maturity-onset diabetes of the young (MODY) is a heterogeneous subtype of NIDDM charac- terized by early onset (usually before age 25 years) autosomal-dominant inheritance, and a primary defect in insulin secretion (1,2). To date, three MODY genes have been identified: hepatocyte nuclear factor- 4 α(HNF-4α/MODY1), glucokinase (GCK/MODY2), and hepatocyte nuclear factor-1α(HNF1_α/MODY3) on chromo- somes 20q, 7p, and 12q, respectively (3-5). Investigation of 67 MODY pedigrees collected in France showed that 63/100 (42 Families) have GCK/MODY2 subtype and 21/100 (14 families) Have HNF1α/MODY3 subtype (4,6,7).