Protein Name aconitase 1
Organism Homo sapiens
Gene ID 48
Gene Symbol

ACO1

UniProt P21399 (ACOC_HUMAN), V9HWB7 (V9HWB7_HUMAN), Q9HBB2 (Q9HBB2_HUMAN)
Relationships Total Number of functionally related compound(s) : 142
Total Number of Articles : 319
Description

aconitase 1

Gene Summary

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 [provided by RefSeq, Jan 2014]

synonyms
  • cytoplasmic aconitate hydratase
  • aconitase 1, soluble
  • aconitate hydratase, cytoplasmic
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Properties