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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 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).
The degree of insulin deficiency is variable. Diabetes can develop from infancy through middle adult life, and some family members who carry the gene remain free of diabetes into later adult life. Most of those who develop diabetes show atrophy of the entire pancreas, with mild or subclinical deficiency of exocrine as well as endocrine function.
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.
The diabetes that accompanies the hearing loss can be similar to Type 1 diabetes or Type 2 diabetes; however, Type 1-like diabetes is the more common form of the two. MIDD has also been associated with a number of other issues including kidney dysfunction, gastrointestinal problems , and cardiomyopathy .
This is an accepted version of this page This is the latest accepted revision, reviewed on 1 March 2025. Group of endocrine diseases characterized by high blood sugar levels This article is about the common insulin disorder. For the urine hyper-production disorder, see Diabetes insipidus. For other uses, see Diabetes (disambiguation). Medical condition Diabetes Universal blue circle symbol for ...
Neonatal diabetes is a genetic disease, caused by genetic variations that were either spontaneously acquired or inherited from one's parents. At least 30 distinct genetic variants can result in neonatal diabetes. [8] The development and treatment of neonatal diabetes will vary based on the particular genetic cause.
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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