HNF4-α

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Name
Hepatocyte nuclear factor 4 receptor α
Symbol
HNF4-α
NRNC Symbol
NR2A1

Overview

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.

Expression

HNF4α is almost exclusively expressed in the gastrointestinal system (stomach, duodenum, jejunum, ileum, colon, gall bladder), liver and kidney, with lower levels in white adipose tissue and pancreas.

View full NURSA HNF4α expression dataset

Diseases

HNF4α dysfunction has been implicated in mature onset diabetes of the youing, (MODY), type I, type II diabetes mellitus, high serum lipid levels, chronic kidney failure and metabolic syndrome.

Phenotypes

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).

View HNF4α Diseases and Phenotypes section


 Nuclear Receptor Pages User Guide (updated Oct 2008)

At a Glance
Phylogeny
Class
HNF4/RXR/TLL/COUP-like receptors
NRNC Group
Hepatocyte nuclear factor 4 (HNF4)

Nomenclature
transcription factor-14 (TCF14); nuclear receptor subfamily 2, group A, member 1 (NR2A1); maturity-onset diabetes of the young (MODY); hepatocyte nuclear factor 4, α (HNF4A); Hepatocyte nuclear factor 4 receptor α (HNF4α)

Primary References
Kritis AA, Argyrokastritis A, Moschonas NK, Power S, Katrakili N, Zannis VI, Cereghini S and Talianidis I (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 and 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 and 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

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