maturity onset diabetes of the young type 3

maturity onset diabetes of the young type 3

An autosomal dominant endocrinopathy (OMIM:600496) of childhood to early adult onset, which is characterised by a primary defect in insulin secretion and frequent insulin-independence at the beginning of the disease.
Molecular pathology
Defects in HNF1A, which encodes a transcription activator that regulates tissue-specific expression of multiple genes, cause maturity onset diabetes of the young type 3.