MitoProteome Database

MT000013

Record overview

MITO ID	MT000013
Gene ID	48
Species	Homo sapiens (Human)
Gene Name	aconitase 1, soluble
Gene Symbol	ACO1
Synonyms	IRP1; ACONS; HEL60; IREB1; IREBP; IREBP1;
Alternate names	cytoplasmic aconitate hydratase; IRE-BP 1; citrate hydro-lyase; iron regulatory protein 1; ferritin repressor protein; epididymis luminal protein 60; aconitate hydratase, cytoplasmic; iron-responsive element binding protein 1; iron-responsive element-binding protein 1;
Chromosome	9
Map Location	9p21.1
EC Number	4.2.1.3
Summary	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]
Orthologs	View orthologs and multiple alignments for ACO1

Proteins

cytoplasmic aconitate hydratase

Refseq ID:	NP_001265281
Protein GI:	507834063
UniProt ID:	P21399
mRNA ID:	NM_001278352
Length:	889
RefSeq Status:

MSNPFAHLAEPLDPVQPGKKFFNLNKLEDSRYGRLPFSIRVLLEAAIRNCDEFLVKKQDIENILHWNVTQHKNIEVPFKPARVILQDFTGVPAVVDFAAM
RDAVKKLGGDPEKINPVCPADLVIDHSIQVDFNRRADSLQKNQDLEFERNRERFEFLKWGSQAFHNMRIIPPGSGIIHQVNLEYLARVVFDQDGYYYPDS
LVGTDSHTTMIDGLGILGWGVGGIEAEAVMLGQPISMVLPQVIGYRLMGKPHPLVTSTDIVLTITKHLRQVGVVGKFVEFFGPGVAQLSIADRATIANMC
PEYGATAAFFPVDEVSITYLVQTGRDEEKLKYIKKYLQAVGMFRDFNDPSQDPDFTQVVELDLKTVVPCCSGPKRPQDKVAVSDMKKDFESCLGAKQGFK
GFQVAPEHHNDHKTFIYDNTEFTLAHGSVVIAAITSCTNTSNPSVMLGAGLLAKKAVDAGLNVMPYIKTSLSPGSGVVTYYLQESGVMPYLSQLGFDVVG
YGCMTCIGNSGPLPEPVVEAITQGDLVAVGVLSGNRNFEGRVHPNTRANYLASPPLVIAYAIAGTIRIDFEKEPLGVNAKGQQVFLKDIWPTRDEIQAVE
RQYVIPGMFKEVYQKIETVNESWNALATPSDKLFFWNSKSTYIKSPPFFENLTLDLQPPKSIVDAYVLLNLGDSVTTDHISPAGNIARNSPAARYLTNRG
LTPREFNSYGSRRGNDAVMARGTFANIRLLNRFLNKQAPQTIHLPSGEILDVFDAAERYQQAGLPLIVLAGKEYGAGSSRDWAAKGPFLLGIKAVLAESY
ERIHRSNLVGMGVIPLEYLPGENADALGLTGQERYTIIIPENLKPQMKVQVKLDTGKTFQAVMRFDTDVELTYFLNGGILNYMIRKMAK

cytoplasmic aconitate hydratase

Refseq ID:	NP_002188
Protein GI:	8659555
UniProt ID:	P21399
mRNA ID:	NM_002197
Length:	889
RefSeq Status:

Protein sequence is identical to GI:507834063 (mRNA isoform)