Protein Aco2

URN urn:agi-llid:50
Connectivity 50
Name Aco2
Description aconitase 2, mitochondrial
Notes The protein encoded by this gene belongs to the aconitase/IPM isomerase family. It is an enzyme that catalyzes the interconversion of citrate to isocitrate via cis-aconitate in the second step of the TCA cycle. This protein is encoded in the nucleus and functions in the mitochondrion. It was found to be one of the mitochondrial matrix proteins that are preferentially degraded by the serine protease 15(PRSS15), also known as Lon protease, after oxidative modification.

Pathway citrate cycle pathway
citric acid cycle pathway

GO Molecular Function lyase activity
iron-sulfur cluster binding
metal ion binding
iron ion binding
3 iron, 4 sulfur cluster binding
4 iron, 4 sulfur cluster binding
aconitate hydratase activity

GO Cellular Component mitochondrion
mitochondrial matrix
nucleus

GO Biological Process metabolic process
generation of precursor metabolites and energy
citrate metabolic process
isocitrate metabolic process
tricarboxylic acid cycle

Ariadne Ontology Tricarboxylic acid cycle

Group Tricarboxylic acid cycle
metabolic process
generation of precursor metabolites and energy
citrate metabolic process
isocitrate metabolic process
tricarboxylic acid cycle
lyase activity
iron-sulfur cluster binding
metal ion binding
iron ion binding
3 iron, 4 sulfur cluster binding
4 iron, 4 sulfur cluster binding
aconitate hydratase activity
mitochondrion
mitochondrial matrix
nucleus

MedScan ID 50

Hugo ID 118

Human chromosome position 22q11.2-q13.31

LocusLink ID 50
11429
79250

Alias ACONM
MGC20605
MGC33908
aconitate hydratase, mitochondrial
citrate hydro-lyase
Aco3
Aco-2
D10Wsu183e
OTTMUSP00000017637
mitochondrial aconitase (nuclear aco2 gene)
ACO2
aconitase 2
aconitase 2, mitochondrial
aconitase II
DNA segment, Chr 10, Wayne State University 183, expressed
LOC115645
mitochondrial aconitase
mitochondrial aconitase 2
mitochondrial aconitase II
mitochondrial Aconitate hydratase
mitochondrial similar to aconitase 2
mitochondrial similar to aconitase II
OTTHUMP00000042146
OTTHUMP00000165920
aconitate hydratase
Aconitate hydratase, mitochondrial precursor
mitochondrial Aconitate hydratase precursor

Organism Homo sapiens
Mus musculus
Rattus norvegicus

GenBank ID NC_000022
NM_001098
NP_001089
NT_011520
NW_001838745
NW_927628
AC_000154
AC_000065
AF093096
AAD19351
AL008582
CAI17931
AL023553
CAI20278
CH471095
EAW60435
EAW60436
U87926
AAC39921
U87927
U87928
U87929
U87930
U87931
U87932
U87933
U87934
U87935
U87936
U87937
U87938
U87939
AA724140
AK293562
BAG57034
AK296260
BAG58973
AK297213
BAG59696
AK301321
BAG62870
AK302691
BAG63920
AK314845
BAG37362
BC014092
AAH14092
BC026196
AAH26196
CR456365
CAG30251
CR536568
CAG38805
CR592345
CR595461
CR596181
CR596278
CR596641
CR613743
CR614581
CR619036
CR621307
U80040
AAB38416
Q71UF1
Q99798
CU012932
CAK54363
CU013220
CAK54662
DQ893674
ABM84600
DQ895836
ABM86762
NC_000081
NM_080633
NP_542364
NT_039621
NW_001030577
AC_000037
AC102103
CH466550
EDL04552
AK143917
BAE25602
AK144207
BAE25770
AK145511
BAE26479
AK150027
BAE29252
AK150954
AK165411
BAE38169
AK182261
AK182718
AK188036
AK189803
AK191827
AK196433
AK196458
AK198224
AK198314
AK198988
AK199021
AK205392
AK205499
AK205787
AK205960
AK206733
AK208429
AK209619
AK209925
AK213382
AK215088
AK217449
AK219343
AK220091
BC004645
AAH04645
BC094462
AAH94462
BI987806
BQ031215
BY732916
CA750686
CO426953
CO429053
Q99KI0
NC_005106
NM_024398
NP_077374
NW_047781
NW_001084859
AC_000075
CH473950
EDM15697
AJ243266
CAC11018
BC061999
AAH61999
Q9ER34

OMIM ID 100850

Mouse chromosome position 15
15 E1

GO ID 0051539
0003994
0005506
0016829
0046872
0006101
0006091
0008152
0006099
0005739
0005634
0051538
0051536
0006102
0005759
0016836

Rat chromosome position 7q34

Swiss-Prot Accession Q99798
Q71UF1
Q99KI0
Q9ER34
Q3UDK9
Q3ULG9
Q3UNH7
Q505P4
Q6P6V3
O75809
Q5JZ41
Q6FHX0
Q8TAQ6

PIR ID S17526
T52543

Unigene ID Hs.643610
Hs.712617
Mm.154581
Rn.43737
Hs.708011

KEGG ID hsa:50
mmu:11429
rno:79250

EC Number 4.2.1.3

Swiss-Prot ID ACON_MOUSE
ACON_RAT
ACON_HUMAN

Cell Localization Mitochondrion
Mitochondria

IPI ID IPI00116074
IPI00421539
IPI00017855
IPI00790739

Homologene ID 856

RGD ID 621360

MGI ID 87880