HEPATOCYTE NUCLEAR FACTOR 4

'HNF4' ('H'epatocyte 'N'uclear 'F'actor 4) is a nuclear receptor protein mostly expressed in the liver, gut, kidney, and pancreatic beta cells that is critical for liver development. In humans, there are two isoforms of NHF4, alpha and gamma encoded by two separate gene and respectively.^[1]

Contents

Ligands

Pathology

See also

References

External links

Ligands

HNF4 was originally classified as an orphan receptor. However HNF4 was found later to be constitutively active by virtue of being continuously bound to a variety of fatty acids.^[2][3] The ligand binding domain of HNF4, as with other nuclear receptors, adopts a canonical alpha helical sandwich fold^[4][5] and interacts with co-activator proteins.^[6]

Pathology

Mutations in the HNF4-α gene have been linked to maturity onset diabetes of the young (MODY).^[7]

See also

★ Hepatocyte nuclear factors

References

1. Cloning and sequencing of cDNAs encoding the human hepatocyte nuclear factor 4 indicate the presence of two isoforms in human liver, Chartier FL, Bossu JP, Laudet V, Fruchart JC, Laine B, , , Gene, 1994
2. Desperately seeking...something, Sladek F, , , Mol Cell, 2002
3. Fatty acid regulation of hepatic gene transcription, Jump DB, Botolin D, Wang Y, Xu J, Christian B, Demeure O, , , J Nutr, 2005
4. Hepatocyte nuclear factor 4 is a transcription factor that constitutively binds fatty acids, Wisely GB, Miller AB, Davis RG, Thornquest AD Jr, Johnson R, Spitzer T, Sefler A, Shearer B, Moore JT, Miller AB, Willson TM, Williams SP, , , Structure, 2002
5. Crystal structure of the HNF4 alpha ligand binding domain in complex with endogenous fatty acid ligand, Dhe-Paganon S, Duda K, Iwamoto M, Chi YI, Shoelson SE, , , J Biol Chem, 2002
6. Structural basis for HNF-4alpha activation by ligand and coactivator binding, Duda K, Chi YI, Shoelson SE, , , J Biol Chem, 2004
7. Molecular mechanisms and clinical pathophysiology of maturity-onset diabetes of the young, Fajans SS, Bell GI, Polonsky KS, , , N Engl J Med, 2001

External links

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