Mody 12 E11.-

MODY is the acronym for "Maturity-onset Diabetes of the Young" and describes a group of autosomal-dominantly inherited, genetically heterogeneous, not always insulin-dependent forms of diabetes. The MODY forms of diabetes are caused by various disorders of beta cell function in the pancreas. The body weight of MODY patients is usually normal. Neither are any of the autoimmune phenomena characteristic of type 1 diabetes observed. Rare are other associated organ dysplasias (eyes, pancreas, intestine).

MODY 12 is produced by monogenic, autosomal-dominantly inherited mutations in the ABCC8 gene (ATP-binding cassette transporter sub-family C member 8) which codes for the sulfonylurea receptor 1 (SUR1).

1. Katanic D et al (2017) A successful transition to sulfonylurea treatment in male infant with neonatal diabetes caused by the novel abcc8 gene mutation and three years follow-up. Diabetes Res Clin Pract 129:59-61.
2. Ovsyannikova AK et al (2016) ABCC8-Related Maturity-Onset Diabetes of the Young (MODY12): Clinical Features and Treatment Perspective. Diabetes Ther 7: 591-600.