Protein Aco1

URN	urn:agi-llid:48
Total Entities	0
Connectivity	328
Name	Aco1
Description	aconitase 1, soluble
Notes	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. Alternative splicing results in multiple transcript variants. [provided by RefSeq, May 2013]

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

GO Cellular Component	cytoplasm
	Golgi apparatus
	cytosol
	endoplasmic reticulum
	mitochondrion
	intracellular membrane-bounded organelle

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

Pathway	doxorubicin response pathway

Group	Tricarboxylic acid cycle
	metabolic process
	tricarboxylic acid cycle
	citrate metabolic process
	post-embryonic development
	intestinal absorption
	cellular iron ion homeostasis
	response to iron(II) ion
	regulation of translation
	regulation of gene expression
	lyase activity
	aconitate hydratase activity
	RNA binding
	mRNA binding
	mRNA 5'-UTR binding
	iron-responsive element binding
	metal ion binding
	iron-sulfur cluster binding
	4 iron, 4 sulfur cluster binding
	cytoplasm
	Golgi apparatus
	cytosol
	endoplasmic reticulum
	mitochondrion
	intracellular membrane-bounded organelle

Cell Localization	Mitochondria
	Cytoplasm

Alias	IRP1
	ACONS
	HEL60
	IREB1
	IREBP
	IREBP1
	RP11-334P12.1
	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
	Aco-1
	AI256519
	RP23-9F23.1
	cytoplasmic aconitase
	Acon1
	Ratireb
	OTTMUSP00000006919
	OTTHUMP00000045233
	OTTHUMP00000021177
	OTTHUMP00000021176
	IRP I
	Iron-responsive element binding protein I
	iron-responsive element-binding protein
	soluble aconitase
	soluble aconitase 1
	soluble aconitase I
	Iron regulatory protein-1
	Ratirebs
	iron-responsive element binding protein
	Aconitase 1 soluble
	Iron regulatory protein I
	ACO I
	ACO1
	Acon I
	aconitase 1
	aconitase 1 variant
	aconitase I
	Aconitase, soluble
	Aconitate hydratase
	IREB I

Mouse chromosome position	4 20.24 cM

OMIM ID	100880

Rat chromosome position	5q22

Hugo ID	117

Human chromosome position	9p21.1

EC Number	4.2.1.3

Homologene ID	1657

MGI ID	87879

RGD ID	2019

MedScan ID	48