Heterozygous variants in the INS gene have been reported in insulin-dependent diabetes mellitus 2 (OMIM:125852), hyperproinsulinemia (OMIM:616214), and maturity-onset diabetes of the young type 10 (MODY 10) (OMIM:613370), all exhibiting autosomal-dominant inheritance. Here, INS is linked to maturity-onset diabetes of the young type 10.