cytoplasmic aconitate hydratase; aconitase; citrate hydro-lyase; iron-responsive element-binding protein 1; IRE-BP 1; iron regulatory protein 1; IRP1; ferritin repressor protein (ACO1, IREB1)

Function

• cytoplasmic form of aconitase may serve very different function from mitochondrial form; i.e. as & RNA-binding protein via the iron-response element
• binds to iron-responsive elements
• binding to the IRE element in ferritin mRNA results in repression of its translation
• binding to transferrin receptor mRNA inhibits degradation of this otherwise rapidly degraded mRNA
• also expresses aconitase activity citrate = isocitrate

Cofactor: binds 1 4Fe-4S cluster per subunit (putative)

Structure

• requires free sulfyhydryl group(s) for activity.
• Fe+2 may promote oxidation of IRE-BP & decreased affinity for IRE
• belongs to the aconitase/IPM isomerase family

Compartment

cytoplasm

More general terms

• aconitase, aconitate-hydratase or citrate hydrolyase
• iron-responsive element [IRE]-binding protein

Additional terms

• iron response element

Database

• UniProt: http://www.uniprot.org/uniprot/P21399.html
• OMIM: https://mirror.omim.org/entry/100880
• OMIM: https://mirror.omim.org/entry/100900
• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=48
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:48; -.