Aconitase 1 (ACO1) Antibody – Rabbit Polyclonal

This gene encodes a member of the aconitase/IPM isomerase protein family. The encoded protein has been identified as a moonlighting protein based on its ability to perform mechanistically distinct functions. Depending on iron levels in the cytosol, the encoded protein can function as either an aconitase enzyme or as an mRNA binding protein. When cellular iron levels are high, the encoded protein functions as an aconitase, an essential enzyme in the TCA cycle that catalyzes the conversion of citrate to isocitrate. When cellular iron levels are low, the encoded protein regulates iron uptake and utilization by binding to iron-responsive elements in the untranslated regions of mRNAs for genes involved in iron metabolism. Disruption of this gene is associated with pulmonary hypertension and polycythemia. [provided by RefSeq, Jan 2014]

IREB1: Iron sensor. Binds a 4Fe-4S cluster and functions as aconitase when cellular iron levels are high. Functions as mRNA binding protein that regulates uptake, sequestration and utilization of iron when cellular iron levels are low. Binds to iron-responsive elements (IRES) in target mRNA species when iron levels are low. Binding of a 4Fe-4S cluster precludes RNA binding. Belongs to the aconitase/IPM isomerase family.

Protein type: Carbohydrate Metabolism - citrate (TCA) cycle; Carbohydrate Metabolism - glyoxylate and dicarboxylate; EC 4.2.1.3; Endoplasmic reticulum; Lyase; RNA-binding; Translation

Chromosomal Location of Human Ortholog: 4 A5|4 20.24 cM

Cellular Component: cytoplasm; cytosol; endoplasmic reticulum; extracellular exosome; Golgi apparatus; intracellular membrane-bound organelle; mitochondrion

Molecular Function: 3 iron, 4 sulfur cluster binding; 4 iron, 4 sulfur cluster binding; aconitate hydratase activity; iron-responsive element binding; iron-sulfur cluster binding; lyase activity; metal ion binding; mRNA 5'-UTR binding; RNA binding

Biological Process: cellular iron ion homeostasis; citrate metabolic process; intestinal absorption; metabolic process; post-embryonic development; regulation of gene expression; regulation of translation; response to iron(II) ion; tricarboxylic acid cycle