Diabetologia
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Multicenter Study
High frequency of mutations in MODY and mitochondrial genes in Scandinavian patients with familial early-onset diabetes.
To investigate the contribution of mutations in maturity-onset diabetes of the young (MODY) and mitochondrial genes to early-onset diabetes with a strong family history of diabetes in a cohort with a high prevalence of Type I (insulin-dependent) diabetes mellitus. ⋯ Among 115 Scandinavian families, mutations in the HNF-1alpha gene represented the most common cause of familial early-onset ( = 40 years) diabetes: MODY3 (5.2 %) more than MODY2 (3.5 %) more than MIDD (2.6 %) more than MODY1 (1.7 %).