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Protein

Aconitate hydratase, mitochondrial

Gene

Aco2

Organism
Mus musculus (Mouse)
Status
Reviewed-Annotation score: -Experimental evidence at protein leveli

Functioni

Catalyzes the isomerization of citrate to isocitrate via cis-aconitate.By similarity

Catalytic activityi

Citrate = isocitrate.By similarity

Cofactori

[4Fe-4S] clusterBy similarityNote: Binds 1 [4Fe-4S] cluster per subunit. Binding of a [3Fe-4S] cluster leads to an inactive enzyme.By similarity

Pathwayi: tricarboxylic acid cycle

This protein is involved in step 2 of the subpathway that synthesizes isocitrate from oxaloacetate.
Proteins known to be involved in the 2 steps of the subpathway in this organism are:
  1. Citrate synthase, mitochondrial (Cs)
  2. Aconitate hydratase, mitochondrial (Aco2)
This subpathway is part of the pathway tricarboxylic acid cycle, which is itself part of Carbohydrate metabolism.
View all proteins of this organism that are known to be involved in the subpathway that synthesizes isocitrate from oxaloacetate, the pathway tricarboxylic acid cycle and in Carbohydrate metabolism.

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Binding sitei99SubstrateBy similarity1
Metal bindingi385Iron-sulfur (4Fe-4S)By similarity1
Metal bindingi448Iron-sulfur (4Fe-4S)By similarity1
Metal bindingi451Iron-sulfur (4Fe-4S)By similarity1
Binding sitei474SubstrateBy similarity1
Binding sitei479SubstrateBy similarity1
Binding sitei607SubstrateBy similarity1

GO - Molecular functioni

GO - Biological processi

Keywordsi

Molecular functionLyase
Biological processTricarboxylic acid cycle
Ligand4Fe-4S, Iron, Iron-sulfur, Metal-binding

Enzyme and pathway databases

ReactomeiR-MMU-71403 Citric acid cycle (TCA cycle)
UniPathwayi
UPA00223;UER00718

Names & Taxonomyi

Protein namesi
Recommended name:
Aconitate hydratase, mitochondrial (EC:4.2.1.3By similarity)
Short name:
Aconitase
Alternative name(s):
Citrate hydro-lyase
Gene namesi
Name:Aco2
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaMyomorphaMuroideaMuridaeMurinaeMusMus
Proteomesi
  • UP000000589 Componenti: Chromosome 15

Organism-specific databases

MGIiMGI:87880 Aco2

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Keywords - Cellular componenti

Mitochondrion

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 27MitochondrionBy similarityAdd BLAST27
ChainiPRO_000000054228 – 780Aconitate hydratase, mitochondrialAdd BLAST753

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei31N6-succinyllysineCombined sources1
Modified residuei50N6-acetyllysine; alternateCombined sources1
Modified residuei50N6-succinyllysine; alternateCombined sources1
Modified residuei138N6-acetyllysine; alternateCombined sources1
Modified residuei138N6-succinyllysine; alternateCombined sources1
Modified residuei144N6-acetyllysine; alternateCombined sources1
Modified residuei144N6-succinyllysine; alternateCombined sources1
Modified residuei233N6-acetyllysine; alternateCombined sources1
Modified residuei233N6-succinyllysine; alternateCombined sources1
Modified residuei411N6-succinyllysineCombined sources1
Modified residuei517N6-acetyllysine; alternateCombined sources1
Modified residuei517N6-succinyllysine; alternateCombined sources1
Modified residuei523N6-acetyllysine; alternateCombined sources1
Modified residuei523N6-succinyllysine; alternateCombined sources1
Modified residuei549N6-succinyllysineCombined sources1
Modified residuei559PhosphoserineBy similarity1
Modified residuei573N6-acetyllysine; alternateBy similarity1
Modified residuei573N6-succinyllysine; alternateCombined sources1
Modified residuei577N6-succinyllysineCombined sources1
Modified residuei591N6-succinyllysineCombined sources1
Modified residuei605N6-acetyllysine; alternateCombined sources1
Modified residuei605N6-succinyllysine; alternateCombined sources1
Modified residuei628N6-succinyllysineCombined sources1
Modified residuei670PhosphoserineCombined sources1
Modified residuei689N6-succinyllysineCombined sources1
Modified residuei723N6-acetyllysine; alternateCombined sources1
Modified residuei723N6-succinyllysine; alternateCombined sources1
Modified residuei730N6-acetyllysine; alternateCombined sources1
Modified residuei730N6-succinyllysine; alternateCombined sources1
Modified residuei736N6-acetyllysineCombined sources1
Modified residuei739N6-acetyllysineCombined sources1
Modified residuei743N6-acetyllysineCombined sources1

Keywords - PTMi

Acetylation, Phosphoprotein

Proteomic databases

EPDiQ99KI0
MaxQBiQ99KI0
PaxDbiQ99KI0
PeptideAtlasiQ99KI0
PRIDEiQ99KI0

2D gel databases

REPRODUCTION-2DPAGEiQ99KI0

PTM databases

CarbonylDBiQ99KI0
iPTMnetiQ99KI0
PhosphoSitePlusiQ99KI0
SwissPalmiQ99KI0

Expressioni

Gene expression databases

BgeeiENSMUSG00000022477 Expressed in 328 organ(s), highest expression level in skeletal muscle tissue
CleanExiMM_ACO2
GenevisibleiQ99KI0 MM

Interactioni

Subunit structurei

Monomer.By similarity

Protein-protein interaction databases

BioGridi197925, 1 interactor
IntActiQ99KI0, 11 interactors
MINTiQ99KI0
STRINGi10090.ENSMUSP00000023116

Structurei

3D structure databases

ProteinModelPortaliQ99KI0
SMRiQ99KI0
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni192 – 194Substrate bindingBy similarity3
Regioni670 – 671Substrate bindingBy similarity2

Sequence similaritiesi

Belongs to the aconitase/IPM isomerase family.Curated

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiKOG0453 Eukaryota
COG1048 LUCA
GeneTreeiENSGT00920000149128
HOGENOMiHOG000224293
HOVERGENiHBG000248
InParanoidiQ99KI0
KOiK01681
OMAiGCIGMGQ
OrthoDBiEOG091G024R
PhylomeDBiQ99KI0
TreeFamiTF300627

Family and domain databases

Gene3Di3.20.19.10, 1 hit
3.30.499.10, 2 hits
3.40.1060.10, 1 hit
InterProiView protein in InterPro
IPR015931 Acnase/IPM_dHydase_lsu_aba_1/3
IPR001030 Acoase/IPM_deHydtase_lsu_aba
IPR015928 Aconitase/3IPM_dehydase_swvl
IPR018136 Aconitase_4Fe-4S_BS
IPR036008 Aconitase_4Fe-4S_dom
IPR015932 Aconitase_dom2
IPR006248 Aconitase_mito-like
IPR000573 AconitaseA/IPMdHydase_ssu_swvl
PfamiView protein in Pfam
PF00330 Aconitase, 1 hit
PF00694 Aconitase_C, 1 hit
PRINTSiPR00415 ACONITASE
SUPFAMiSSF53732 SSF53732, 1 hit
TIGRFAMsiTIGR01340 aconitase_mito, 1 hit
PROSITEiView protein in PROSITE
PS00450 ACONITASE_1, 1 hit
PS01244 ACONITASE_2, 1 hit

Sequence (1+)i

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

This entry has 1 described isoform and 4 potential isoforms that are computationally mapped.iShow all

Q99KI0-1 [UniParc]FASTAAdd to basket
« Hide
        10         20         30         40         50
MAPYSLLVTR LQKALGVRQY HVASVLCQRA KVAMSHFEPS EYIRYDLLEK
60 70 80 90 100
NINIVRKRLN RPLTLSEKIV YGHLDDPANQ EIERGKTYLR LRPDRVAMQD
110 120 130 140 150
ATAQMAMLQF ISSGLPKVAV PSTIHCDHLI EAQVGGEKDL RRAKDINQEV
160 170 180 190 200
YNFLATAGAK YGVGFWRPGS GIIHQIILEN YAYPGVLLIG TDSHTPNGGG
210 220 230 240 250
LGGICIGVGG ADAVDVMAGI PWELKCPKVI GVKLTGSLSG WTSPKDVILK
260 270 280 290 300
VAGILTVKGG TGAIVEYHGP GVDSISCTGM ATICNMGAEI GATTSVFPYN
310 320 330 340 350
HRMKKYLSKT GRTDIANLAE EFKDHLVPDP GCQYDQVIEI NLNELKPHIN
360 370 380 390 400
GPFTPDLAHP VADVGTVAEK EGWPLDIRVG LIGSCTNSSY EDMGRSAAVA
410 420 430 440 450
KQALAHGLKC KSQFTITPGS EQIRATIERD GYAQILRDVG GIVLANACGP
460 470 480 490 500
CIGQWDRKDI KKGEKNTIVT SYNRNFTGRN DANPETHAFV TSPEIVTALA
510 520 530 540 550
IAGTLKFNPE TDFLTGKDGK KFKLEAPDAD ELPRSDFDPG QDTYQHPPKD
560 570 580 590 600
SSGQRVDVSP TSQRLQLLEP FDKWDGKDLE DLQILIKVKG KCTTDHISAA
610 620 630 640 650
GPWLKFRGHL DNISNNLLIG AINIENGKAN SVRNAVTQEF GPVPDTARYY
660 670 680 690 700
KKHGIRWVVI GDENYGEGSS REHAALEPRH LGGRAIITKS FARIHETNLK
710 720 730 740 750
KQGLLPLTFA DPSDYNKIHP VDKLTIQGLK DFAPGKPLKC VIKHPNGTQE
760 770 780
TILLNHTFNE TQIEWFRAGS ALNRMKELQQ
Length:780
Mass (Da):85,464
Last modified:June 1, 2001 - v1
Checksum:i9B515846E875D581
GO

Computationally mapped potential isoform sequencesi

There are 4 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
A0A2R8W744A0A2R8W744_MOUSE
Aconitate hydratase, mitochondrial
Aco2
194Annotation score:
A0A2R8VJW0A0A2R8VJW0_MOUSE
Aconitate hydratase, mitochondrial
Aco2
62Annotation score:
A0A2R8VJM1A0A2R8VJM1_MOUSE
Aconitate hydratase, mitochondrial
Aco2
77Annotation score:
A0A2R8VHM8A0A2R8VHM8_MOUSE
Aconitate hydratase, mitochondrial
Aco2
58Annotation score:

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti7 – 8LV → P in BAE25770 (PubMed:16141072).Curated2
Sequence conflicti618L → F in AAH94462 (PubMed:15489334).Curated1
Sequence conflicti758F → L in BAE29252 (PubMed:16141072).Curated1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AK143917 mRNA Translation: BAE25602.1
AK144207 mRNA Translation: BAE25770.1
AK145511 mRNA Translation: BAE26479.1
AK150027 mRNA Translation: BAE29252.1
AK165411 mRNA Translation: BAE38169.1
BC004645 mRNA Translation: AAH04645.1
BC094462 mRNA Translation: AAH94462.1
CCDSiCCDS27675.1
RefSeqiNP_542364.1, NM_080633.2
UniGeneiMm.154581

Genome annotation databases

EnsembliENSMUST00000023116; ENSMUSP00000023116; ENSMUSG00000022477
GeneIDi11429
KEGGimmu:11429
UCSCiuc007wxp.1 mouse

Similar proteinsi

Entry informationi

Entry nameiACON_MOUSE
AccessioniPrimary (citable) accession number: Q99KI0
Secondary accession number(s): Q3UDK9
, Q3ULG9, Q3UNH7, Q505P4
Entry historyiIntegrated into UniProtKB/Swiss-Prot: February 1, 2005
Last sequence update: June 1, 2001
Last modified: September 12, 2018
This is version 139 of the entry and version 1 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Keywords - Technical termi

Complete proteome, Direct protein sequencing, Reference proteome
UniProt is an ELIXIR core data resource
Main funding by: National Institutes of Health

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