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Title: Hnf4  
Author: World Heritage Encyclopedia
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Subject: Epigenetics, Nuclear receptor
Publisher: World Heritage Encyclopedia


hepatocyte nuclear factor 4 alpha
Symbol HNF4A
Alt. symbols TCF14, MODY, MODY1
Entrez HUGO OMIM PDB 1M7W (RefSeq UniProt Locus q12-20q13.1
hepatocyte nuclear factor 4 gamma
Symbol HNF4G
Entrez HUGO OMIM PDB 1LV2 (RefSeq UniProt Locus q21-q22

HNF4 (Hepatocyte Nuclear Factor 4) is a


HNF4 was originally classified as an orphan receptor that exhibits constitutive transactivation activity apparently by being continuously bound to a variety of fatty acids.[2][3] The existence of a ligand for HNF4 has been somewhat controversial, but linoleic acid (LA) has been identified as the endogenous ligand of native HNF4 expressed in mouse liver; the binding of LA to HNF4 is reversible.[4]

The ligand binding domain of HNF4, as with other nuclear receptors, adopts a canonical alpha helical sandwich fold[5][6] and interacts with co-activator proteins.[7]

HNF4 binds to the consensus sequence AGGTCAaAGGTCA in order to activate transcription.


Mutations in the HNF4A gene have been linked to maturity onset diabetes of the young 1 (MODY1).[8]

See also


External links

  • Medical Subject Headings (MeSH)
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