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MODY 2 or GCK-MODY is a form of maturity-onset diabetes of the young. [1] It is due to any of several mutations in the GCK gene on human chromosome 7 for glucokinase. [2] Glucokinase serves as the glucose sensor for the pancreatic beta cell. Normal glucokinase triggers insulin secretion as the glucose exceeds about 90 mg/dl (5 mM).
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.
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Renal cysts and diabetes syndrome (RCAD), also known as MODY 5 or HNF1B-MODY, is a form of maturity onset diabetes of the young. Presentation
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.
MODY 1 or HNF4A-MODY is a form of maturity onset diabetes of the young. MODY 1 is due to a loss-of-function mutation in the HNF4A ( MODY1 ) gene on chromosome 12 . This gene codes for hepatocyte nuclear factor 4-alpha (HNF4-α) protein also known as transcription factor 14 (TCF14).
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.
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