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Protein

Citrate synthase, mitochondrial

Gene

CS

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

Functioni

Catalytic activityi

Acetyl-CoA + H2O + oxaloacetate = citrate + CoA.PROSITE-ProRule annotation

Pathwayi: tricarboxylic acid cycle

This protein is involved in step 1 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
Active sitei301PROSITE-ProRule annotation1
Active sitei347PROSITE-ProRule annotation1
Active sitei402PROSITE-ProRule annotation1

GO - Molecular functioni

  • citrate (Si)-synthase activity Source: UniProtKB
  • poly(A) RNA binding Source: UniProtKB

GO - Biological processi

Complete GO annotation...

Keywords - Molecular functioni

Transferase

Keywords - Biological processi

Tricarboxylic acid cycle

Enzyme and pathway databases

BioCyciMetaCyc:ENSG00000062485-MONOMER.
ZFISH:ENSG00000062485-MONOMER.
BRENDAi2.3.3.1. 2681.
ReactomeiR-HSA-1268020. Mitochondrial protein import.
R-HSA-71403. Citric acid cycle (TCA cycle).
UniPathwayiUPA00223; UER00717.

Names & Taxonomyi

Protein namesi
Recommended name:
Citrate synthase, mitochondrial (EC:2.3.3.1)
Alternative name(s):
Citrate (Si)-synthase
Gene namesi
Name:CS
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 12

Organism-specific databases

HGNCiHGNC:2422. CS.

Subcellular locationi

GO - Cellular componenti

  • extracellular exosome Source: UniProtKB
  • mitochondrial matrix Source: UniProtKB
  • mitochondrion Source: UniProtKB
  • nucleus Source: UniProtKB
Complete GO annotation...

Keywords - Cellular componenti

Mitochondrion

Pathology & Biotechi

Organism-specific databases

DisGeNETi1431.
OpenTargetsiENSG00000062485.
PharmGKBiPA26928.

Polymorphism and mutation databases

BioMutaiCS.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 27MitochondrionAdd BLAST27
ChainiPRO_000000547128 – 466Citrate synthase, mitochondrialAdd BLAST439

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei57N6-succinyllysineBy similarity1
Modified residuei76N6-acetyllysine; alternateBy similarity1
Modified residuei76N6-succinyllysine; alternateBy similarity1
Modified residuei103N6-succinyllysineBy similarity1
Modified residuei193N6-succinyllysineBy similarity1
Modified residuei321N6-acetyllysine; alternateBy similarity1
Modified residuei321N6-succinyllysine; alternateBy similarity1
Modified residuei327N6-acetyllysine; alternateCombined sources1
Modified residuei327N6-succinyllysine; alternateBy similarity1
Modified residuei375N6-acetyllysine; alternateCombined sources1
Modified residuei375N6-succinyllysine; alternateBy similarity1
Modified residuei382N6-acetyllysineCombined sources1
Modified residuei393N6-acetyllysine; alternateCombined sources1
Modified residuei393N6-succinyllysine; alternateBy similarity1
Modified residuei395N6,N6,N6-trimethyllysineBy similarity1
Modified residuei450N6-succinyllysineBy similarity1
Modified residuei459N6-acetyllysine; alternateBy similarity1
Modified residuei459N6-succinyllysine; alternateBy similarity1

Keywords - PTMi

Acetylation, Methylation

Proteomic databases

EPDiO75390.
MaxQBiO75390.
PaxDbiO75390.
PeptideAtlasiO75390.
PRIDEiO75390.
TopDownProteomicsiO75390.

PTM databases

iPTMnetiO75390.
PhosphoSitePlusiO75390.
SwissPalmiO75390.

Expressioni

Gene expression databases

BgeeiENSG00000062485.
CleanExiHS_CS.
ExpressionAtlasiO75390. baseline and differential.
GenevisibleiO75390. HS.

Organism-specific databases

HPAiCAB075750.
CAB075751.
CAB075752.
CAB075753.
CAB075754.
HPA038460.
HPA038461.

Interactioni

Subunit structurei

Homodimer.By similarity

Protein-protein interaction databases

BioGridi107818. 27 interactors.
IntActiO75390. 6 interactors.
MINTiMINT-1162839.
STRINGi9606.ENSP00000342056.

Structurei

3D structure databases

ProteinModelPortaliO75390.
SMRiO75390.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Belongs to the citrate synthase family.Curated

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiKOG2617. Eukaryota.
COG0372. LUCA.
GeneTreeiENSGT00390000006813.
HOGENOMiHOG000130831.
HOVERGENiHBG005336.
InParanoidiO75390.
KOiK01647.
OMAiDYIWKTL.
OrthoDBiEOG091G068F.
PhylomeDBiO75390.
TreeFamiTF300398.

Family and domain databases

Gene3Di1.10.580.10. 1 hit.
InterProiIPR016142. Citrate_synth-like_lrg_a-sub.
IPR002020. Citrate_synthase.
IPR019810. Citrate_synthase_AS.
IPR010109. Citrate_synthase_euk.
[Graphical view]
PANTHERiPTHR11739. PTHR11739. 1 hit.
PfamiPF00285. Citrate_synt. 1 hit.
[Graphical view]
PRINTSiPR00143. CITRTSNTHASE.
SUPFAMiSSF48256. SSF48256. 1 hit.
TIGRFAMsiTIGR01793. cit_synth_euk. 1 hit.
PROSITEiPS00480. CITRATE_SYNTHASE. 1 hit.
[Graphical view]

Sequencei

Sequence statusi: Complete.

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

O75390-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MALLTAAARL LGTKNASCLV LAARHASASS TNLKDILADL IPKEQARIKT
60 70 80 90 100
FRQQHGKTVV GQITVDMMYG GMRGMKGLVY ETSVLDPDEG IRFRGFSIPE
110 120 130 140 150
CQKLLPKAKG GEEPLPEGLF WLLVTGHIPT EEQVSWLSKE WAKRAALPSH
160 170 180 190 200
VVTMLDNFPT NLHPMSQLSA AVTALNSESN FARAYAQGIS RTKYWELIYE
210 220 230 240 250
DSMDLIAKLP CVAAKIYRNL YREGSGIGAI DSNLDWSHNF TNMLGYTDHQ
260 270 280 290 300
FTELTRLYLT IHSDHEGGNV SAHTSHLVGS ALSDPYLSFA AAMNGLAGPL
310 320 330 340 350
HGLANQEVLV WLTQLQKEVG KDVSDEKLRD YIWNTLNSGR VVPGYGHAVL
360 370 380 390 400
RKTDPRYTCQ REFALKHLPN DPMFKLVAQL YKIVPNVLLE QGKAKNPWPN
410 420 430 440 450
VDAHSGVLLQ YYGMTEMNYY TVLFGVSRAL GVLAQLIWSR ALGFPLERPK
460
SMSTEGLMKF VDSKSG
Length:466
Mass (Da):51,712
Last modified:January 4, 2005 - v2
Checksum:i459CB29C0BA06997
GO

Sequence cautioni

The sequence CAE45911 differs from that shown. Intron retention.Curated

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti55H → R in CAE45911 (PubMed:17974005).Curated1
Sequence conflicti127H → C in AAC25560 (PubMed:9809442).Curated1
Sequence conflicti183R → Q in AAC25560 (PubMed:9809442).Curated1
Sequence conflicti187Q → R in AAC25560 (PubMed:9809442).Curated1
Sequence conflicti203M → V in AAC25560 (PubMed:9809442).Curated1
Sequence conflicti222R → W in AAC25560 (PubMed:9809442).Curated1
Sequence conflicti255T → M in AAC25560 (PubMed:9809442).Curated1
Sequence conflicti282L → F in AAQ13428 (PubMed:12549038).Curated1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF047042 mRNA. Translation: AAC25560.1.
AF053631 mRNA. Translation: AAQ13428.1.
AK074956 mRNA. Translation: BAC11314.1.
BX640838 mRNA. Translation: CAE45911.1. Sequence problems.
BC000105 mRNA. Translation: AAH00105.3.
BC010106 mRNA. Translation: AAH10106.1.
BC072016 mRNA. Translation: AAH72016.1.
BT007414 mRNA. Translation: AAP36082.1.
CCDSiCCDS8913.1.
RefSeqiNP_004068.2. NM_004077.2.
UniGeneiHs.743252.

Genome annotation databases

EnsembliENST00000351328; ENSP00000342056; ENSG00000062485.
GeneIDi1431.
KEGGihsa:1431.
UCSCiuc001skr.2. human.

Cross-referencesi

Web resourcesi

Wikipedia

Citrate synthase entry

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF047042 mRNA. Translation: AAC25560.1.
AF053631 mRNA. Translation: AAQ13428.1.
AK074956 mRNA. Translation: BAC11314.1.
BX640838 mRNA. Translation: CAE45911.1. Sequence problems.
BC000105 mRNA. Translation: AAH00105.3.
BC010106 mRNA. Translation: AAH10106.1.
BC072016 mRNA. Translation: AAH72016.1.
BT007414 mRNA. Translation: AAP36082.1.
CCDSiCCDS8913.1.
RefSeqiNP_004068.2. NM_004077.2.
UniGeneiHs.743252.

3D structure databases

ProteinModelPortaliO75390.
SMRiO75390.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi107818. 27 interactors.
IntActiO75390. 6 interactors.
MINTiMINT-1162839.
STRINGi9606.ENSP00000342056.

PTM databases

iPTMnetiO75390.
PhosphoSitePlusiO75390.
SwissPalmiO75390.

Polymorphism and mutation databases

BioMutaiCS.

Proteomic databases

EPDiO75390.
MaxQBiO75390.
PaxDbiO75390.
PeptideAtlasiO75390.
PRIDEiO75390.
TopDownProteomicsiO75390.

Protocols and materials databases

DNASUi1431.
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000351328; ENSP00000342056; ENSG00000062485.
GeneIDi1431.
KEGGihsa:1431.
UCSCiuc001skr.2. human.

Organism-specific databases

CTDi1431.
DisGeNETi1431.
GeneCardsiCS.
HGNCiHGNC:2422. CS.
HPAiCAB075750.
CAB075751.
CAB075752.
CAB075753.
CAB075754.
HPA038460.
HPA038461.
MIMi118950. gene.
neXtProtiNX_O75390.
OpenTargetsiENSG00000062485.
PharmGKBiPA26928.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG2617. Eukaryota.
COG0372. LUCA.
GeneTreeiENSGT00390000006813.
HOGENOMiHOG000130831.
HOVERGENiHBG005336.
InParanoidiO75390.
KOiK01647.
OMAiDYIWKTL.
OrthoDBiEOG091G068F.
PhylomeDBiO75390.
TreeFamiTF300398.

Enzyme and pathway databases

UniPathwayiUPA00223; UER00717.
BioCyciMetaCyc:ENSG00000062485-MONOMER.
ZFISH:ENSG00000062485-MONOMER.
BRENDAi2.3.3.1. 2681.
ReactomeiR-HSA-1268020. Mitochondrial protein import.
R-HSA-71403. Citric acid cycle (TCA cycle).

Miscellaneous databases

ChiTaRSiCS. human.
GenomeRNAii1431.
PROiO75390.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000062485.
CleanExiHS_CS.
ExpressionAtlasiO75390. baseline and differential.
GenevisibleiO75390. HS.

Family and domain databases

Gene3Di1.10.580.10. 1 hit.
InterProiIPR016142. Citrate_synth-like_lrg_a-sub.
IPR002020. Citrate_synthase.
IPR019810. Citrate_synthase_AS.
IPR010109. Citrate_synthase_euk.
[Graphical view]
PANTHERiPTHR11739. PTHR11739. 1 hit.
PfamiPF00285. Citrate_synt. 1 hit.
[Graphical view]
PRINTSiPR00143. CITRTSNTHASE.
SUPFAMiSSF48256. SSF48256. 1 hit.
TIGRFAMsiTIGR01793. cit_synth_euk. 1 hit.
PROSITEiPS00480. CITRATE_SYNTHASE. 1 hit.
[Graphical view]
ProtoNetiSearch...

Entry informationi

Entry nameiCISY_HUMAN
AccessioniPrimary (citable) accession number: O75390
Secondary accession number(s): Q71UT9
, Q7KZH0, Q96FZ8, Q9BWN8
Entry historyi
Integrated into UniProtKB/Swiss-Prot: July 15, 1999
Last sequence update: January 4, 2005
Last modified: November 2, 2016
This is version 157 of the entry and version 2 of the sequence. [Complete history]
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

Miscellaneousi

Miscellaneous

Citrate synthase is found in nearly all cells capable of oxidative metabolism.

Keywords - Technical termi

Complete proteome, Direct protein sequencing, Reference proteome

Documents

  1. Human chromosome 12
    Human chromosome 12: entries, gene names and cross-references to MIM
  2. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  3. PATHWAY comments
    Index of metabolic and biosynthesis pathways
  4. 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.