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Nuclear Receptor Signaling Atlas
A research resource for the nuclear receptor signaling community
HNF4-α
Last updated January 31, 2014
Overview
NURSA Name
Hepatocyte nuclear factor 4 receptor α
NURSA Symbol
HNF4-α
Synonyms
HNF4-α
Description
HNF4α is a member of the HNF4 orphan subfamily of the nuclear receptor superfamily of transcription factors that is required for hepatocyte differentiation and development of the liver. It has been shown to constitutively bind fatty acids. HNF4α is almost exclusively expressed in the gastrointestinal system (stomach, duodenum, jejunum, ileum, colon, and gall bladder), liver and kidney, with lower levels in white adipose tissue and pancreas. HNF4α dysfunction has been implicated in mature onset diabetes of the young, (MODY), type I, type II diabetes mellitus, high serum lipid levels, chronic kidney failure and metabolic syndrome. Targeted disruption (knockout) of HNF4α results in defects in embryonic development, homeostasis and metabolism, the liver and biliary system and the central nervous system (CNS).
Original References:
Kritis AA,Argyrokastritis A,Moschonas NK,Power S,Katrakili N,Zannis VI, et al. (1996) Isolation and characterization of a third isoform of human hepatocyte nuclear factor 4. Gene 173 275-80 View Abstract | View Pubmed
Chartier FL,Bossu JP,Laudet V,Fruchart JC,Laine B, (1994) Cloning and sequencing of cDNAs encoding the human hepatocyte nuclear factor 4 indicate the presence of two isoforms in human liver. Gene 147 269-72 View Abstract | View Pubmed
Sladek FM,Zhong WM,Lai E,Darnell JE, (1990) Liver-enriched transcription factor HNF-4 is a novel member of the steroid hormone receptor superfamily. Genes Dev. 4 2353-65 View Abstract | View Pubmed
View Pubmed

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