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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)LengthDescriptionGraphical viewFeature identifierActions
Binding sitei99 – 991SubstrateBy similarity
Metal bindingi385 – 3851Iron-sulfur (4Fe-4S)By similarity
Metal bindingi448 – 4481Iron-sulfur (4Fe-4S)By similarity
Metal bindingi451 – 4511Iron-sulfur (4Fe-4S)By similarity
Binding sitei474 – 4741SubstrateBy similarity
Binding sitei479 – 4791SubstrateBy similarity
Binding sitei607 – 6071SubstrateBy similarity

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)LengthDescriptionGraphical viewFeature identifierActions
Transit peptidei1 – 2727MitochondrionBy similarityAdd
BLAST
Chaini28 – 780753Aconitate hydratase, mitochondrialPRO_0000000542Add
BLAST

Amino acid modifications

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Modified residuei28 – 281Pyrrolidone carboxylic acidBy similarity
Modified residuei31 – 311N6-succinyllysineCombined sources
Modified residuei50 – 501N6-acetyllysine; alternateCombined sources
Modified residuei50 – 501N6-succinyllysine; alternateCombined sources
Modified residuei138 – 1381N6-acetyllysine; alternateCombined sources
Modified residuei138 – 1381N6-succinyllysine; alternateCombined sources
Modified residuei144 – 1441N6-acetyllysine; alternateCombined sources
Modified residuei144 – 1441N6-succinyllysine; alternateCombined sources
Cross-linki144 – 144Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in ubiquitin); alternate1 Publication
Modified residuei233 – 2331N6-acetyllysine; alternateCombined sources
Modified residuei233 – 2331N6-succinyllysine; alternateCombined sources
Modified residuei411 – 4111N6-succinyllysineCombined sources
Modified residuei517 – 5171N6-acetyllysine; alternateCombined sources
Modified residuei517 – 5171N6-succinyllysine; alternateCombined sources
Modified residuei523 – 5231N6-acetyllysine; alternateCombined sources
Modified residuei523 – 5231N6-succinyllysine; alternateCombined sources
Modified residuei549 – 5491N6-succinyllysineCombined sources
Modified residuei559 – 5591PhosphoserineBy similarity
Modified residuei573 – 5731N6-acetyllysine; alternateBy similarity
Modified residuei573 – 5731N6-succinyllysine; alternateCombined sources
Modified residuei577 – 5771N6-succinyllysineCombined sources
Modified residuei591 – 5911N6-succinyllysineCombined sources
Modified residuei605 – 6051N6-acetyllysine; alternateCombined sources
Modified residuei605 – 6051N6-succinyllysine; alternateCombined sources
Modified residuei628 – 6281N6-succinyllysineCombined sources
Modified residuei670 – 6701PhosphoserineCombined sources
Modified residuei689 – 6891N6-succinyllysineCombined sources
Modified residuei723 – 7231N6-acetyllysine; alternateCombined sources
Modified residuei723 – 7231N6-succinyllysine; alternateCombined sources
Modified residuei730 – 7301N6-acetyllysine; alternateCombined sources
Modified residuei730 – 7301N6-succinyllysine; alternateCombined sources
Modified residuei736 – 7361N6-acetyllysineCombined sources
Modified residuei739 – 7391N6-acetyllysineCombined sources
Modified residuei743 – 7431N6-acetyllysineCombined sources

Post-translational modificationi

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

Keywords - PTMi

Acetylation, Isopeptide bond, Phosphoprotein, Pyrrolidone carboxylic acid, Ubl conjugation

Proteomic databases

EPDiQ99KI0.
MaxQBiQ99KI0.
PaxDbiQ99KI0.
PeptideAtlasiQ99KI0.
PRIDEiQ99KI0.

2D gel databases

REPRODUCTION-2DPAGEQ99KI0.

PTM databases

iPTMnetiQ99KI0.
PhosphoSiteiQ99KI0.
SwissPalmiQ99KI0.

Expressioni

Gene expression databases

BgeeiENSMUSG00000022477.
CleanExiMM_ACO2.
GenevisibleiQ99KI0. MM.

Interactioni

Subunit structurei

Monomer.By similarity

Protein-protein interaction databases

BioGridi197925. 1 interaction.
IntActiQ99KI0. 9 interactions.
MINTiMINT-1842680.
STRINGi10090.ENSMUSP00000023116.

Structurei

3D structure databases

ProteinModelPortaliQ99KI0.
SMRiQ99KI0. Positions 29-780.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Region

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Regioni192 – 1943Substrate bindingBy similarity
Regioni670 – 6712Substrate bindingBy similarity

Sequence similaritiesi

Belongs to the aconitase/IPM isomerase family.Curated

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiKOG0453. Eukaryota.
COG1048. LUCA.
GeneTreeiENSGT00840000129913.
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)LengthDescriptionGraphical viewFeature identifierActions
Sequence conflicti7 – 82LV → P in BAE25770 (PubMed:16141072).Curated
Sequence conflicti618 – 6181L → F in AAH94462 (PubMed:15489334).Curated
Sequence conflicti758 – 7581F → L in BAE29252 (PubMed:16141072).Curated

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. Positions 29-780.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi197925. 1 interaction.
IntActiQ99KI0. 9 interactions.
MINTiMINT-1842680.
STRINGi10090.ENSMUSP00000023116.

PTM databases

iPTMnetiQ99KI0.
PhosphoSiteiQ99KI0.
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.
GeneTreeiENSGT00840000129913.
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: September 7, 2016
This is version 126 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.