View source for MODY 4

{{more citations needed|date=March 2018}}
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'''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 (human)|chromosome 13]]. Pdx-1 is a transcription factor vital to the development of the embryonic pancreas. Even in adults it continues to play a role in the regulation and expression of genes for insulin, GLUT2, glucokinase, and [[somatostatin]].

MODY 4 is so rare that only a single family has been well-studied. A child born with [[pancreatic agenesis]] (absence of the pancreas) was found to be [[homozygous]] for Pdx-1 mutations. A number of older relatives who were heterozygous had mild hyperglycemia or diabetes. None were severely insulin-deficient and all were controlled with either diet or [[oral hypoglycemic agent]]s.

== References ==
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{{Disease of the pancreas and glucose metabolism}}
{{Transcription factor/coregulator deficiencies}}

<ref>{{cite journal|last1=Stoffers|first1=Doris A.|last2=Ferrer|first2=Jorge|last3=L. Clarke|first3=William|last4=Habener|first4=Joel|title=Early-onset type-II diabetes mellitus (MODY4) linked to IPF1|journal=Nature Genetics|date=1997|volume=17|pages=138–139|doi=10.1038/ng1097-138|ref=}}</ref>

[[Category:Types of diabetes]]
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