Biology:ACO1

Short description: Protein-coding gene in the species Homo sapiens

Generic protein structure example

Aconitase 1, soluble is a protein that in humans is encoded by the ACO1 gene.

Function

The protein encoded by this gene is a bifunctional, cytosolic protein that functions as an essential enzyme in the TCA cycle and interacts with mRNA to control the levels of iron inside cells. When cellular iron levels are high, this protein binds to a 4Fe-4S cluster and functions as an aconitase. Aconitases are iron-sulfur proteins that function to catalyze the conversion of citrate to isocitrate. When cellular iron levels are low, the protein binds to iron-responsive elements (IREs), which are stem-loop structures found in the 5' UTR of ferritin mRNA, and in the 3' UTR of transferrin receptor mRNA. When the protein binds to IRE, it results in repression of translation of ferritin mRNA, and inhibition of degradation of the otherwise rapidly degraded transferrin receptor mRNA. The encoded protein has been identified as a moonlighting protein based on its ability to perform mechanistically distinct functions. Alternative splicing results in multiple transcript variants.

External links

• Human ACO1 genome location and ACO1 gene details page in the UCSC Genome Browser.

Further reading

• "Excess capacity of the iron regulatory protein system". The Journal of Biological Chemistry 282 (34): 24650–9. Aug 2007. doi:10.1074/jbc.M703167200. PMID 17604281. 
• "Folding and turnover of human iron regulatory protein 1 depend on its subcellular localization". The FEBS Journal 274 (4): 1083–92. Feb 2007. doi:10.1111/j.1742-4658.2007.05657.x. PMID 17244191. 
• "Demonstration and characterization of the iron regulatory protein in human brain". Journal of Neurochemistry 67 (2): 838–44. Aug 1996. doi:10.1046/j.1471-4159.1996.67020838.x. PMID 8764614. 
• "Inhibition of an iron-responsive element/iron regulatory protein-1 complex by ATP binding and hydrolysis". The FEBS Journal 274 (12): 3108–19. Jun 2007. doi:10.1111/j.1742-4658.2007.05843.x. PMID 17521334. 
• "Iron-dependent degradation of apo-IRP1 by the ubiquitin-proteasome pathway". Molecular and Cellular Biology 27 (7): 2423–30. Apr 2007. doi:10.1128/MCB.01111-06. PMID 17242182. 
• "[The expression of TfR1 mRNA and IRP1 mRNA in the placenta from different maternal iron status]". Zhonghua Xue Ye Xue Za Zhi = Zhonghua Xueyexue Zazhi 28 (4): 255–8. Apr 2007. PMID 17877204. 
• "The connectivity map links iron regulatory protein-1-mediated inhibition of hypoxia-inducible factor-2a translation to the anti-inflammatory 15-deoxy-delta12,14-prostaglandin J2". Cancer Research 70 (8): 3071–9. Apr 2010. doi:10.1158/0008-5472.CAN-09-2877. PMID 20354189. 
• "HBx modulates iron regulatory protein 1-mediated iron metabolism via reactive oxygen species". Virus Research 133 (2): 167–77. May 2008. doi:10.1016/j.virusres.2007.12.014. PMID 18262302. 
• "Iron accumulation and iron-regulatory protein activity in human hepatoma (HepG2) cells". Molecular and Cellular Biochemistry 265 (1–2): 37–45. Oct 2004. doi:10.1023/b:mcbi.0000044313.19574.c6. PMID 15543932. 

This article incorporates text from the United States National Library of Medicine, which is in the public domain.