NP_002188.1
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NCBI GenBank Nucleotide #
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UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
98,399 Da
NCBI Official Full Name
cytoplasmic aconitate hydratase
NCBI Official Synonym Full Names
aconitase 1
NCBI Official Synonym Symbols
IRP1; ACONS; HEL60; IREB1; IREBP; IREBP1 [Similar Products]
NCBI Protein Information
cytoplasmic aconitate hydratase
UniProt Protein Name
Cytoplasmic aconitate hydratase
UniProt Synonym Protein Names
Citrate hydro-lyase; Ferritin repressor protein; Iron regulatory protein 1; IRP1; Iron-responsive element-binding protein 1; IRE-BP 1
UniProt Synonym Gene Names
UniProt Entry Name
ACOC_HUMAN
NCBI Summary for ACO1
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]
UniProt Comments for ACO1
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: EC 4.2.1.3; Lyase; Carbohydrate Metabolism - glyoxylate and dicarboxylate; Carbohydrate Metabolism - citrate (TCA) cycle; Endoplasmic reticulum; Translation; RNA-binding
Chromosomal Location of Human Ortholog: 9p21.1
Cellular Component: cytoplasm; cytosol; endoplasmic reticulum; Golgi apparatus; mitochondrion
Molecular Function: 4 iron, 4 sulfur cluster binding; aconitate hydratase activity; iron-responsive element binding; metal ion binding; protein binding; RNA binding
Biological Process: cellular iron ion homeostasis; citrate metabolic process; intestinal absorption; post-embryonic development; regulation of translation; response to iron(II) ion; tricarboxylic acid cycle
Research Articles on ACO1
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Products associated with ACO1 blocking peptide
Pathways associated with ACO1 blocking peptide
Diseases associated with ACO1 blocking peptide
Organs/Tissues associated with ACO1 blocking peptide
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