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ACO1

Function

Bifunctional iron sensor that switches between 2 activities depending on iron availability (PubMed:1281544, PubMed:1946430, PubMed:8041788). Iron deprivation, promotes its mRNA binding activity through which it regulates the expression of genes involved in iron uptake, sequestration and utilization (PubMed:1281544, PubMed:1946430, PubMed:23891004, PubMed:8041788). Binds to iron-responsive elements (IRES) in the untranslated region of target mRNAs preventing for instance the translation of ferritin and aminolevulinic acid synthase and stabilizing the transferrin receptor mRNA (PubMed:1281544, PubMed:1946430, PubMed:23891004, PubMed:8041788).

Conversely, when cellular iron levels are high, binds a 4Fe-4S cluster which precludes RNA binding activity and promotes the aconitase activity, the isomerization of citrate to isocitrate via cis-aconitate.

Sequence Similarities

Belongs to the aconitase/IPM isomerase family.

Cellular localization

Alternative names

IREB1, ACO1, Cytoplasmic aconitate hydratase, Aconitase, Citrate hydro-lyase, Ferritin repressor protein, Iron regulatory protein 1, Iron-responsive element-binding protein 1, IRP1, IRE-BP 1

swissprot:P21399 omim:100880 entrezGene:48