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Protein

Aconitate hydratase, mitochondrial

Gene

Aco2

Organism
Mus musculus (Mouse)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

Functioni

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

Catalytic activityi

Citrate = isocitrate.

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

Complete GO annotation...

Keywords - Molecular functioni

Lyase

Keywords - Biological processi

Tricarboxylic acid cycle

Keywords - Ligandi

4Fe-4S, Iron, Iron-sulfur, Metal-binding

Enzyme and pathway databases

ReactomeiR-MMU-71403. Citric acid cycle (TCA cycle).
UniPathwayiUPA00223; UER00718.

Names & Taxonomyi

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

Organism-specific databases

MGIiMGI:87880. Aco2.

Subcellular locationi

GO - Cellular componenti

  • mitochondrion Source: MGI
  • myelin sheath Source: UniProtKB
  • nucleus Source: MGI
Complete GO annotation...

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 residuei28Pyrrolidone carboxylic acidBy similarity1
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

Post-translational modificationi

Acetylation of Lys-50 is observed in liver mitochondria from fasted mice but not from fed mice.

Keywords - PTMi

Acetylation, Phosphoprotein, Pyrrolidone carboxylic acid

Proteomic databases

EPDiQ99KI0.
MaxQBiQ99KI0.
PaxDbiQ99KI0.
PeptideAtlasiQ99KI0.
PRIDEiQ99KI0.

2D gel databases

REPRODUCTION-2DPAGEQ99KI0.

PTM databases

iPTMnetiQ99KI0.
PhosphoSitePlusiQ99KI0.
SwissPalmiQ99KI0.

Expressioni

Gene expression databases

BgeeiENSMUSG00000022477.
CleanExiMM_ACO2.
GenevisibleiQ99KI0. MM.

Interactioni

Subunit structurei

Monomer.By similarity

Protein-protein interaction databases

BioGridi197925. 1 interactor.
IntActiQ99KI0. 9 interactors.
MINTiMINT-1842680.
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.
GeneTreeiENSGT00860000133751.
HOGENOMiHOG000224293.
HOVERGENiHBG000248.
InParanoidiQ99KI0.
KOiK01681.
OMAiPDLGHPI.
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.
InterProiIPR015931. Acnase/IPM_dHydase_lsu_aba_1/3.
IPR015937. Acoase/IPM_deHydtase.
IPR001030. Acoase/IPM_deHydtase_lsu_aba.
IPR015928. Aconitase/3IPM_dehydase_swvl.
IPR015932. Aconitase/IPMdHydase_lsu_aba_2.
IPR018136. Aconitase_4Fe-4S_BS.
IPR006248. Aconitase_mito-like.
IPR000573. AconitaseA/IPMdHydase_ssu_swvl.
[Graphical view]
PANTHERiPTHR11670. PTHR11670. 1 hit.
PfamiPF00330. Aconitase. 1 hit.
PF00694. Aconitase_C. 1 hit.
[Graphical view]
PRINTSiPR00415. ACONITASE.
SUPFAMiSSF52016. SSF52016. 1 hit.
SSF53732. SSF53732. 1 hit.
TIGRFAMsiTIGR01340. aconitase_mito. 1 hit.
PROSITEiPS00450. ACONITASE_1. 1 hit.
PS01244. ACONITASE_2. 1 hit.
[Graphical view]

Sequencei

Sequence statusi: Complete.

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

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

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.

Cross-referencesi

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.

3D structure databases

ProteinModelPortaliQ99KI0.
SMRiQ99KI0.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi197925. 1 interactor.
IntActiQ99KI0. 9 interactors.
MINTiMINT-1842680.
STRINGi10090.ENSMUSP00000023116.

PTM databases

iPTMnetiQ99KI0.
PhosphoSitePlusiQ99KI0.
SwissPalmiQ99KI0.

2D gel databases

REPRODUCTION-2DPAGEQ99KI0.

Proteomic databases

EPDiQ99KI0.
MaxQBiQ99KI0.
PaxDbiQ99KI0.
PeptideAtlasiQ99KI0.
PRIDEiQ99KI0.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

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

Organism-specific databases

CTDi50.
MGIiMGI:87880. Aco2.

Phylogenomic databases

eggNOGiKOG0453. Eukaryota.
COG1048. LUCA.
GeneTreeiENSGT00860000133751.
HOGENOMiHOG000224293.
HOVERGENiHBG000248.
InParanoidiQ99KI0.
KOiK01681.
OMAiPDLGHPI.
OrthoDBiEOG091G024R.
PhylomeDBiQ99KI0.
TreeFamiTF300627.

Enzyme and pathway databases

UniPathwayiUPA00223; UER00718.
ReactomeiR-MMU-71403. Citric acid cycle (TCA cycle).

Miscellaneous databases

ChiTaRSiAco2. mouse.
PROiQ99KI0.
SOURCEiSearch...

Gene expression databases

BgeeiENSMUSG00000022477.
CleanExiMM_ACO2.
GenevisibleiQ99KI0. MM.

Family and domain databases

Gene3Di3.20.19.10. 1 hit.
3.30.499.10. 2 hits.
3.40.1060.10. 1 hit.
InterProiIPR015931. Acnase/IPM_dHydase_lsu_aba_1/3.
IPR015937. Acoase/IPM_deHydtase.
IPR001030. Acoase/IPM_deHydtase_lsu_aba.
IPR015928. Aconitase/3IPM_dehydase_swvl.
IPR015932. Aconitase/IPMdHydase_lsu_aba_2.
IPR018136. Aconitase_4Fe-4S_BS.
IPR006248. Aconitase_mito-like.
IPR000573. AconitaseA/IPMdHydase_ssu_swvl.
[Graphical view]
PANTHERiPTHR11670. PTHR11670. 1 hit.
PfamiPF00330. Aconitase. 1 hit.
PF00694. Aconitase_C. 1 hit.
[Graphical view]
PRINTSiPR00415. ACONITASE.
SUPFAMiSSF52016. SSF52016. 1 hit.
SSF53732. SSF53732. 1 hit.
TIGRFAMsiTIGR01340. aconitase_mito. 1 hit.
PROSITEiPS00450. ACONITASE_1. 1 hit.
PS01244. ACONITASE_2. 1 hit.
[Graphical view]
ProtoNetiSearch...

Entry informationi

Entry nameiACON_MOUSE
AccessioniPrimary (citable) accession number: Q99KI0
Secondary accession number(s): Q3UDK9
, Q3ULG9, Q3UNH7, Q505P4
Entry historyi
Integrated into UniProtKB/Swiss-Prot: February 1, 2005
Last sequence update: June 1, 2001
Last modified: November 30, 2016
This is version 129 of the entry and version 1 of the sequence. [Complete history]
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Keywords - Technical termi

Complete proteome, Direct protein sequencing, Reference proteome

Documents

  1. MGD cross-references
    Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot
  2. PATHWAY comments
    Index of metabolic and biosynthesis pathways
  3. SIMILARITY comments
    Index of protein domains and families

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.