Protein: P21399

UniprotKB AC UniprotKB ID Gene name Full name Species Curated set
P21399 (Uniprot) ACOC_HUMAN ACO1 Cytoplasmic aconitate hydratase human Yes
Uniprot: 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.Catalyzes the isomerization of citrate to isocitrate via cis-aconitate. more..
Dissimilar Functions Manual Curation

Function 1: Functions as aconitase, citrate to isocitrate. Binds sulfur-iron clusters. Condition: high cellular iron concentration.

Function 2: Functions as mRNA binding protein by binding several IRE in the 3' untranslated region of the transferrin receptor mRNA and controls the degradation of transferrin receptor mRNA. Condition: low cellular iron concentration.

Publications (PMID): 1334546, 1444477, 18261896, 15877277

Biological Processes
Module ID (MoonGO) GO ID (BP) GO Name
225 GO:0016070 RNA metabolic process
225 GO:0010467 gene expression
Cellular Components
Module ID (MoonGO) GO ID (CC) GO Name
225 GO:0031981 nuclear lumen
GO ID (BP) GO Name Evidence Code (GO EC)
GO:0006099 tricarboxylic acid cycle IEA
GO:0006101 citrate metabolic process IDA
GO:0006417 regulation of translation IEA
GO:0006879 cellular iron ion homeostasis TAS
GO:0009791 post-embryonic development IEA
GO:0010040 response to iron(II) ion IDA
GO:0050892 intestinal absorption IEA
GO ID (CC) GO Name Evidence Code (GO EC)
GO:0005737 cytoplasm NAS
GO:0005739 mitochondrion IDA
GO:0005783 endoplasmic reticulum IDA
GO:0005794 Golgi apparatus IDA
GO:0005829 cytosol TAS
GO:0070062 extracellular exosome IDA
No pairs of PrOnto dissimilar CC GO terms found.
PMID Article Title
1738601 Expression of active iron regulatory factor from a full-length human cDNA by in vitro transcription/translation.
1903202 Homology between IRE-BP, a regulatory RNA-binding protein, aconitase, and isopropylmalate isomerase.
1946430 A regulated RNA binding protein also possesses aconitase activity.
2172968 Cloning of the cDNA encoding an RNA regulatory protein -- the human iron-responsive element-binding protein.
8041788 The bifunctional iron-responsive element binding protein/cytosolic aconitase: the role of active-site residues in ligand binding and regulation.
15164053 DNA sequence and analysis of human chromosome 9.
15489334 The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC).
16407072 Crystal structure of human iron regulatory protein 1 as cytosolic aconitase.
19762596 Control of iron homeostasis by an iron-regulated ubiquitin ligase.
19762597 An E3 ligase possessing an iron responsive hemerythrin domain is a regulator of iron homeostasis.
20053667 Molecular control of the cytosolic aconitase/IRP1 switch by extramitochondrial frataxin.
21269460 Initial characterization of the human central proteome.
23033978 Diagnostic exome sequencing in persons with severe intellectual disability.
24275569 An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome.
No results found.
Domain Name Domain ID Source
AconitaseA/IPMdHydase_ssu_swvl IPR000573 InterPro
Acoase/IPM_deHydtase_lsu_aba IPR001030 InterPro
Aconitase/IRP2 IPR006249 InterPro
Aconitase/3IPM_dehydase_swvl IPR015928 InterPro
Acnase/IPM_dHydase_lsu_aba_1/3 IPR015931 InterPro
Aconitase_4Fe-4S_BS IPR018136 InterPro
IRE-BP1 IPR029784 InterPro
Aconitase_4Fe-4S_dom IPR036008 InterPro
Aconitase PF00330 Pfam
Aconitase_C PF00694 Pfam
ACONITASE PR00415 PRINTS
ACONITASE_1 PS00450 PROSITE
ACONITASE_2 PS01244 PROSITE
PTHR11670 PTHR11670 PANTHER
PTHR11670:SF32 PTHR11670:SF32 PANTHER
S26403 S26403 PIR
SSF53732 SSF53732 SUPFAM
aconitase_1 TIGR01341 TIGRFAMs