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Maturity-onset diabetes of the young (MODY) refers to any of several hereditary forms of diabetes mellitus caused by mutations in an autosomal dominant gene disrupting insulin production. [1] Along with neonatal diabetes , MODY is a form of the conditions known as monogenic diabetes.
MODY 3 or HNF1A-MODY is a form of maturity-onset diabetes of the young. It is caused by mutations of the HNF1-alpha gene, a homeobox gene on human chromosome 12 . This is the most common type of MODY in populations with European ancestry, [ 1 ] accounting for about 70% of all cases in Europe.
MODY 4 or PDX1-MODY is a form of maturity onset diabetes of the young. MODY 4 arises from mutations of the PDX1 homeobox gene on chromosome 13 . Pdx-1 is a transcription factor vital to the development of the embryonic pancreas.
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MODY 6 or NEUROD1-MODY is a form of maturity onset diabetes of the young. MODY 6 arises from mutations of the gene for the transcription factor referred to as neurogenic differentiation 1 . The gene is on chromosome 2 in a region of the p arm known as IDDM7 because it includes genes affecting susceptibility to diabetes mellitus type 1 (NeuroD1 ...