Protein Information

General Information
MoonProt ID234
First appeared in release1.0
Name(s)Aconitase Cytoplasmic aconitate hydratase Citrate hydro-lyase Ferritin repressor protein Iron regulatory protein 1 IRP1 Iron-responsive element-binding protein 1 IRE-BP 1 Gene Name: ACO1
UniProt IDP21399 (ACOC_HUMAN), Reviewed
GO termsGO:0006099 tricarboxylic acid cycle GO:0006101 citrate metabolic process GO:0006417 regulation of translation GO:0006879 cellular iron ion homeostasis GO:0008152 metabolic process GO:0009791 post-embryonic development GO:0010040 response to iron(II) ion GO:0010468 regulation of gene expression GO:0050892 intestinal absorption GO:0003723 RNA binding GO:0003994 aconitate hydratase activity GO:0005515 protein binding GO:0016829 lyase activity GO:0030350 iron-responsive element binding GO:0046872 metal ion binding GO:0051536 iron-sulfur cluster binding GO:0051539 4 iron, 4 sulfur cluster binding GO:0005737 cytoplasm GO:0005739 mitochondrion GO:0005783 endoplasmic reticulum GO:0005794 Golgi apparatus GO:0005829 cytosol GO:0070062 extracellular vesicular exosome
Organisms for which functions have been demonstratedHomo sapiens (human, a mammal)
Sequence length889
FASTA sequence>sp|P21399|ACOC_HUMAN Cytoplasmic aconitate hydratase OS=Homo sapiens GN=ACO1 PE=1 SV=3 MSNPFAHLAEPLDPVQPGKKFFNLNKLEDSRYGRLPFSIRVLLEAAIRNCDEFLVKKQDIENILHWNVTQHKNIEVPFKPARVILQDFTGVPAVVDFAAMRDAVKKLGGDPEKINPVCPADLVIDHSIQVDFNRRADSLQKNQDLEFERNRERFEFLKWGSQAFHNMRIIPPGSGIIHQVNLEYLARVVFDQDGYYYPDSLVGTDSHTTMIDGLGILGWGVGGIEAEAVMLGQPISMVLPQVIGYRLMGKPHPLVTSTDIVLTITKHLRQVGVVGKFVEFFGPGVAQLSIADRATIANMCPEYGATAAFFPVDEVSITYLVQTGRDEEKLKYIKKYLQAVGMFRDFNDPSQDPDFTQVVELDLKTVVPCCSGPKRPQDKVAVSDMKKDFESCLGAKQGFKGFQVAPEHHNDHKTFIYDNTEFTLAHGSVVIAAITSCTNTSNPSVMLGAGLLAKKAVDAGLNVMPYIKTSLSPGSGVVTYYLQESGVMPYLSQLGFDVVGYGCMTCIGNSGPLPEPVVEAITQGDLVAVGVLSGNRNFEGRVHPNTRANYLASPPLVIAYAIAGTIRIDFEKEPLGVNAKGQQVFLKDIWPTRDEIQAVERQYVIPGMFKEVYQKIETVNESWNALATPSDKLFFWNSKSTYIKSPPFFENLTLDLQPPKSIVDAYVLLNLGDSVTTDHISPAGNIARNSPAARYLTNRGLTPREFNSYGSRRGNDAVMARGTFANIRLLNRFLNKQAPQTIHLPSGEILDVFDAAERYQQAGLPLIVLAGKEYGAGSSRDWAAKGPFLLGIKAVLAESYERIHRSNLVGMGVIPLEYLPGENADALGLTGQERYTIIIPENLKPQMKVQVKLDTGKTFQAVMRFDTDVELTYFLNGGILNYMIRKMAK
Structure Information
PDB ID2B3X
Quaternary structure
SCOPThe "swivelling" beta/beta/alpha domain, Aconitase iron-sulfur domain
CATH3.30.499.10, 3.40.1060.10, 6.10.190.10, 3.20.19.10
TM Helix Predictionno TM helices
DisProt AnnotationNot in DisProt
Predicted Disorder Regions1-15, 395-413, 685-687, 888-889
Connections to Disease
OMIM ID
Function 1
Function descriptionaconitase, enzyme 4Fe-4S cluster in active site when cellular iron levels are high Citrate <=> isocitrate Citric acid cycle
References for functionPhilpott CC, Klausner RD, Rouault TA. The bifunctional iron-responsive element binding protein/cytosolic aconitase: the role of active-site residues in ligand binding and regulation. Proc Natl Acad Sci U S A. 1994 Jul 19. PMID: 8041788
E.C. number4.2.1.3
Location of functional site(s)
Cellular location of functioncytoplasm
Comments
Function 2
Function descriptioniron responsive element binding protein when cellular iron concentrations are low, loses 4Fe-4S cluster and binds to iron-responsive elements (IRES) in mRNA that encodes proteins that are involved in iron uptake and use
References for functionPhilpott CC, Klausner RD, Rouault TA. The bifunctional iron-responsive element binding protein/cytosolic aconitase: the role of active-site residues in ligand binding and regulation. Proc Natl Acad Sci U S A. 1994 Jul 19. PMID: 8041788
E.C. numberN/A
Location of functional site(s)
Cellular location of functioncytoplasm
Comments