Familial Patterns of Inheritance of Diabetes Mellitus. Maturity-Onset Type Diabetes of Young People (MODY)

Abstract

Since 1960, we have reported in prospective studies that nonprogressive, maturity-onset type diabetes can be found in children, adolescents and young adults (1). We used the term maturity-onset type diabetes of the young in 1964, and reported that it occurred in a strong familial association. Since 1966, the occurence of maturity-onset type diabetes of the young has been amply confirmed by many investigators. In 1974, Tattersall reported three pedigrees with this type of diabetes and proposed that it was inherited in an autosomal dominant fashion and that it was distinct from JOD (2). In 1976, Tattersall and Fajans first used the abbreviation of MODY for maturity-onset diabetes of young people, gave data for 26 families with MODY, showed that there was a difference in the pattern of inheritence between classic juvenile-onset type diabetes and MODY, and provided further evidence for autosomal dominant inheritence of MODY (3). Barbosa et al. described data for two kindreds with MODY and suggested an association with HLA-A3 and BW15 in one of the two pedigrees (4), a finding not duplicated in pedigrees of other investigators, suggesting genetic heterogeneity in MODY.