maturity onset diabetes of the young type 4

maturity onset diabetes of the young type 4

An autosomal dominant endocrinopathy (OMIM:606392) 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 PDX1, which encodes a transcription activator that regulates tissue-specific expression of multiple genes, cause maturity onset diabetes of the young type 4.