Fig. 4.

Family pedigree of patients with MODY and mitochondrial diabetes. Family pedigree of patients with MODY (A: HNF1A mutations and B: PDX1 mutation) and mitochondrial diabetes (C). Filled squares and circles, diabetes; open squares and circles, normal glucose tolerance; squares and circles with a question mark, not tested for diabetes. N denotes wild-type allele, and M denotes mutations (A, both p.R263H and p.H577D mutations; B, p.P101L mutation; C, mitochondrial A3243G mutation). Age of patient and age at onset of diabetes in years is written below each symbol. Proband is depicted with an arrow. MODY, maturity-onset diabetes in the young.