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Protein

Succinate--CoA ligase [ADP-forming] subunit beta, mitochondrial

Gene

SUCLA2

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

Functioni

ATP-specific succinyl-CoA synthetase functions in the citric acid cycle (TCA), coupling the hydrolysis of succinyl-CoA to the synthesis of ATP and thus represents the only step of substrate-level phosphorylation in the TCA (PubMed:15877282). The beta subunit provides nucleotide specificity of the enzyme and binds the substrate succinate, while the binding sites for coenzyme A and phosphate are found in the alpha subunit (By similarity).UniRule annotation1 Publication

Catalytic activityi

ATP + succinate + CoA = ADP + phosphate + succinyl-CoA.UniRule annotation1 Publication

Cofactori

Mg2+UniRule annotationNote: Binds 1 Mg2+ ion per subunit.UniRule annotation

Pathwayi: tricarboxylic acid cycle

This protein is involved in step 1 of the subpathway that synthesizes succinate from succinyl-CoA (ligase route).UniRule annotation1 Publication
Proteins known to be involved in this subpathway in this organism are:
  1. Succinate--CoA ligase [ADP/GDP-forming] subunit alpha, mitochondrial (SUCLG1), Succinate--CoA ligase [GDP-forming] subunit beta, mitochondrial (SUCLG2), Succinate--CoA ligase [ADP-forming] subunit beta, mitochondrial (SUCLA2)
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 succinate from succinyl-CoA (ligase route), the pathway tricarboxylic acid cycle and in Carbohydrate metabolism.

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sitei94Important for substrate specificityUniRule annotation1
Binding sitei98ATPUniRule annotation1
Sitei162Important for substrate specificityUniRule annotation1
Metal bindingi258MagnesiumUniRule annotation1
Metal bindingi272MagnesiumUniRule annotation1
Binding sitei323Substrate; shared with subunit alphaUniRule annotation1

Regions

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Nucleotide bindingi105 – 107ATPUniRule annotation3

GO - Molecular functioni

  • ATP binding Source: UniProtKB-KW
  • metal ion binding Source: InterPro
  • succinate-CoA ligase (ADP-forming) activity Source: UniProtKB

GO - Biological processi

  • succinate metabolic process Source: Ensembl
  • succinyl-CoA metabolic process Source: ProtInc
  • succinyl-CoA pathway Source: UniProtKB
  • tricarboxylic acid cycle Source: Reactome
Complete GO annotation...

Keywords - Molecular functioni

Ligase

Keywords - Biological processi

Tricarboxylic acid cycle

Keywords - Ligandi

ATP-binding, Magnesium, Metal-binding, Nucleotide-binding

Enzyme and pathway databases

BioCyciMetaCyc:ENSG00000136143-MONOMER.
ZFISH:ENSG00000136143-MONOMER.
BRENDAi6.2.1.5. 2681.
ReactomeiR-HSA-71403. Citric acid cycle (TCA cycle).
UniPathwayiUPA00223; UER00999.

Names & Taxonomyi

Protein namesi
Recommended name:
Succinate--CoA ligase [ADP-forming] subunit beta, mitochondrialUniRule annotation (EC:6.2.1.5UniRule annotation1 Publication)
Alternative name(s):
ATP-specific succinyl-CoA synthetase subunit betaUniRule annotation
Short name:
A-SCSUniRule annotation
Succinyl-CoA synthetase beta-A chainUniRule annotation
Short name:
SCS-betaAUniRule annotation
Gene namesi
Name:SUCLA2UniRule annotation
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 13

Organism-specific databases

HGNCiHGNC:11448. SUCLA2.

Subcellular locationi

GO - Cellular componenti

  • extracellular exosome Source: UniProtKB
  • mitochondrial matrix Source: Reactome
  • mitochondrion Source: UniProtKB
  • myelin sheath Source: Ensembl
Complete GO annotation...

Keywords - Cellular componenti

Mitochondrion

Pathology & Biotechi

Involvement in diseasei

Mitochondrial DNA depletion syndrome 5 (MTDPS5)4 Publications
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionA disorder due to mitochondrial dysfunction. It is characterized by infantile onset of hypotonia, neurologic deterioration, a hyperkinetic-dystonic movement disorder, external ophthalmoplegia, deafness, variable renal tubular dysfunction, and mild methylmalonic aciduria in some patients.
See also OMIM:612073
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_046215118G → R in MTDPS5. 1 PublicationCorresponds to variant rs121908537dbSNPEnsembl.1
Natural variantiVAR_070123251D → N in MTDPS5. 1 PublicationCorresponds to variant rs397515462dbSNPEnsembl.1
Natural variantiVAR_046216284R → C in MTDPS5. 1 PublicationCorresponds to variant rs121908538dbSNPEnsembl.1

Keywords - Diseasei

Disease mutation

Organism-specific databases

DisGeNETi8803.
MalaCardsiSUCLA2.
MIMi612073. phenotype.
OpenTargetsiENSG00000136143.
Orphaneti1933. Mitochondrial DNA depletion syndrome, encephalomyopathic form with methylmalonic aciduria.
PharmGKBiPA36245.

Chemistry databases

DrugBankiDB00139. Succinic acid.

Polymorphism and mutation databases

BioMutaiSUCLA2.
DMDMi94730427.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 52MitochondrionCombined sourcesAdd BLAST52
ChainiPRO_000003335253 – 463Succinate--CoA ligase [ADP-forming] subunit beta, mitochondrialUniRule annotationAdd BLAST411

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei78N6-acetyllysineCombined sources1
Modified residuei84PhosphotyrosineCombined sources1
Modified residuei88N6-acetyllysine; alternateBy similarity1
Modified residuei88N6-succinyllysine; alternateBy similarity1
Modified residuei129N6-acetyllysineBy similarity1
Modified residuei139N6-acetyllysineBy similarity1
Modified residuei143N6-acetyllysineCombined sources1
Modified residuei216N6-acetyllysineBy similarity1
Modified residuei279PhosphoserineBy similarity1
Modified residuei341PhosphothreonineBy similarity1
Modified residuei368N6-acetyllysineBy similarity1

Keywords - PTMi

Acetylation, Phosphoprotein

Proteomic databases

EPDiQ9P2R7.
MaxQBiQ9P2R7.
PaxDbiQ9P2R7.
PeptideAtlasiQ9P2R7.
PRIDEiQ9P2R7.

PTM databases

iPTMnetiQ9P2R7.
PhosphoSitePlusiQ9P2R7.
SwissPalmiQ9P2R7.

Expressioni

Tissue specificityi

Widely expressed. Not expressed in liver and lung.1 Publication

Gene expression databases

BgeeiENSG00000136143.
CleanExiHS_SUCLA2.
ExpressionAtlasiQ9P2R7. baseline and differential.
GenevisibleiQ9P2R7. HS.

Organism-specific databases

HPAiHPA039435.
HPA039536.
HPA061528.

Interactioni

Subunit structurei

Heterodimer of an alpha and a beta subunit. The beta subunit determines specificity for ATP (By similarity). Interacts with ALAS2 (PubMed:14643893).UniRule annotation1 Publication

Protein-protein interaction databases

BioGridi114331. 51 interactors.
IntActiQ9P2R7. 16 interactors.
MINTiMINT-3078782.
STRINGi9606.ENSP00000367923.

Structurei

3D structure databases

ProteinModelPortaliQ9P2R7.
SMRiQ9P2R7.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini61 – 288ATP-graspUniRule annotationAdd BLAST228

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni380 – 382Substrate binding; shared with subunit alphaUniRule annotation3

Sequence similaritiesi

Belongs to the succinate/malate CoA ligase beta subunit family. ATP-specific subunit beta subfamily.UniRule annotation
Contains 1 ATP-grasp domain.UniRule annotation

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiKOG2799. Eukaryota.
COG0045. LUCA.
GeneTreeiENSGT00390000010170.
HOVERGENiHBG055555.
InParanoidiQ9P2R7.
KOiK01900.
OMAiYIESGCD.
OrthoDBiEOG091G07T9.
PhylomeDBiQ9P2R7.
TreeFamiTF300624.

Family and domain databases

Gene3Di3.30.1490.20. 1 hit.
3.30.470.20. 1 hit.
3.40.50.261. 1 hit.
HAMAPiMF_00558. Succ_CoA_beta. 1 hit.
MF_03220. Succ_CoA_betaA_euk. 1 hit.
InterProiIPR011761. ATP-grasp.
IPR013650. ATP-grasp_succ-CoA_synth-type.
IPR013815. ATP_grasp_subdomain_1.
IPR013816. ATP_grasp_subdomain_2.
IPR005811. CoA_ligase.
IPR017866. Succ-CoA_synthase_bsu_CS.
IPR005809. Succ_CoA_synthase_bsu.
IPR016102. Succinyl-CoA_synth-like.
[Graphical view]
PANTHERiPTHR11815. PTHR11815. 1 hit.
PfamiPF08442. ATP-grasp_2. 1 hit.
PF00549. Ligase_CoA. 1 hit.
[Graphical view]
PIRSFiPIRSF001554. SucCS_beta. 1 hit.
SUPFAMiSSF52210. SSF52210. 1 hit.
TIGRFAMsiTIGR01016. sucCoAbeta. 1 hit.
PROSITEiPS50975. ATP_GRASP. 1 hit.
PS01217. SUCCINYL_COA_LIG_3. 1 hit.
[Graphical view]

Sequences (2)i

Sequence statusi: Complete.

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

This entry describes 2 isoformsi produced by alternative splicing. AlignAdd to basket

Isoform 1 (identifier: Q9P2R7-1) [UniParc]FASTAAdd to basket

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

« Hide

        10         20         30         40         50
MAASMFYGRL VAVATLRNHR PRTAQRAAAQ VLGSSGLFNN HGLQVQQQQQ
60 70 80 90 100
RNLSLHEYMS MELLQEAGVS VPKGYVAKSP DEAYAIAKKL GSKDVVIKAQ
110 120 130 140 150
VLAGGRGKGT FESGLKGGVK IVFSPEEAKA VSSQMIGKKL FTKQTGEKGR
160 170 180 190 200
ICNQVLVCER KYPRREYYFA ITMERSFQGP VLIGSSHGGV NIEDVAAESP
210 220 230 240 250
EAIIKEPIDI EEGIKKEQAL QLAQKMGFPP NIVESAAENM VKLYSLFLKY
260 270 280 290 300
DATMIEINPM VEDSDGAVLC MDAKINFDSN SAYRQKKIFD LQDWTQEDER
310 320 330 340 350
DKDAAKANLN YIGLDGNIGC LVNGAGLAMA TMDIIKLHGG TPANFLDVGG
360 370 380 390 400
GATVHQVTEA FKLITSDKKV LAILVNIFGG IMRCDVIAQG IVMAVKDLEI
410 420 430 440 450
KIPVVVRLQG TRVDDAKALI ADSGLKILAC DDLDEAARMV VKLSEIVTLA
460
KQAHVDVKFQ LPI
Length:463
Mass (Da):50,317
Last modified:May 2, 2006 - v3
Checksum:i1E1651728AF3B5CD
GO
Isoform 2 (identifier: Q9P2R7-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     26-47: Missing.

Note: No experimental confirmation available.
Show »
Length:441
Mass (Da):48,040
Checksum:iB0B5E3CA063BEAD1
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti40N → D in BAA91939 (PubMed:14702039).Curated1
Sequence conflicti203I → V in BAA91939 (PubMed:14702039).Curated1
Sequence conflicti323N → S in BAA91703 (PubMed:14702039).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_04621413V → M.Corresponds to variant rs35201084dbSNPEnsembl.1
Natural variantiVAR_046215118G → R in MTDPS5. 1 PublicationCorresponds to variant rs121908537dbSNPEnsembl.1
Natural variantiVAR_013459199S → T.3 PublicationsCorresponds to variant rs7320366dbSNPEnsembl.1
Natural variantiVAR_070123251D → N in MTDPS5. 1 PublicationCorresponds to variant rs397515462dbSNPEnsembl.1
Natural variantiVAR_046216284R → C in MTDPS5. 1 PublicationCorresponds to variant rs121908538dbSNPEnsembl.1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_00629226 – 47Missing in isoform 2. 1 PublicationAdd BLAST22

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AB035863 mRNA. Translation: BAA92873.1.
AK001458 mRNA. Translation: BAA91703.1.
AK001847 mRNA. Translation: BAA91939.1.
AK315513 mRNA. Translation: BAG37894.1.
AL157369 Genomic DNA. Translation: CAI15149.1.
AL157369 Genomic DNA. Translation: CAI15153.1.
CH471075 Genomic DNA. Translation: EAX08777.1.
BC027587 mRNA. Translation: AAH27587.1.
AF058953 mRNA. Translation: AAC64396.1.
CCDSiCCDS9406.1. [Q9P2R7-1]
RefSeqiNP_003841.1. NM_003850.2. [Q9P2R7-1]
UniGeneiHs.743361.

Genome annotation databases

EnsembliENST00000378654; ENSP00000367923; ENSG00000136143. [Q9P2R7-1]
GeneIDi8803.
KEGGihsa:8803.
UCSCiuc001vbs.3. human. [Q9P2R7-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AB035863 mRNA. Translation: BAA92873.1.
AK001458 mRNA. Translation: BAA91703.1.
AK001847 mRNA. Translation: BAA91939.1.
AK315513 mRNA. Translation: BAG37894.1.
AL157369 Genomic DNA. Translation: CAI15149.1.
AL157369 Genomic DNA. Translation: CAI15153.1.
CH471075 Genomic DNA. Translation: EAX08777.1.
BC027587 mRNA. Translation: AAH27587.1.
AF058953 mRNA. Translation: AAC64396.1.
CCDSiCCDS9406.1. [Q9P2R7-1]
RefSeqiNP_003841.1. NM_003850.2. [Q9P2R7-1]
UniGeneiHs.743361.

3D structure databases

ProteinModelPortaliQ9P2R7.
SMRiQ9P2R7.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi114331. 51 interactors.
IntActiQ9P2R7. 16 interactors.
MINTiMINT-3078782.
STRINGi9606.ENSP00000367923.

Chemistry databases

DrugBankiDB00139. Succinic acid.

PTM databases

iPTMnetiQ9P2R7.
PhosphoSitePlusiQ9P2R7.
SwissPalmiQ9P2R7.

Polymorphism and mutation databases

BioMutaiSUCLA2.
DMDMi94730427.

Proteomic databases

EPDiQ9P2R7.
MaxQBiQ9P2R7.
PaxDbiQ9P2R7.
PeptideAtlasiQ9P2R7.
PRIDEiQ9P2R7.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000378654; ENSP00000367923; ENSG00000136143. [Q9P2R7-1]
GeneIDi8803.
KEGGihsa:8803.
UCSCiuc001vbs.3. human. [Q9P2R7-1]

Organism-specific databases

CTDi8803.
DisGeNETi8803.
GeneCardsiSUCLA2.
GeneReviewsiSUCLA2.
HGNCiHGNC:11448. SUCLA2.
HPAiHPA039435.
HPA039536.
HPA061528.
MalaCardsiSUCLA2.
MIMi603921. gene.
612073. phenotype.
neXtProtiNX_Q9P2R7.
OpenTargetsiENSG00000136143.
Orphaneti1933. Mitochondrial DNA depletion syndrome, encephalomyopathic form with methylmalonic aciduria.
PharmGKBiPA36245.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG2799. Eukaryota.
COG0045. LUCA.
GeneTreeiENSGT00390000010170.
HOVERGENiHBG055555.
InParanoidiQ9P2R7.
KOiK01900.
OMAiYIESGCD.
OrthoDBiEOG091G07T9.
PhylomeDBiQ9P2R7.
TreeFamiTF300624.

Enzyme and pathway databases

UniPathwayiUPA00223; UER00999.
BioCyciMetaCyc:ENSG00000136143-MONOMER.
ZFISH:ENSG00000136143-MONOMER.
BRENDAi6.2.1.5. 2681.
ReactomeiR-HSA-71403. Citric acid cycle (TCA cycle).

Miscellaneous databases

ChiTaRSiSUCLA2. human.
GeneWikiiSUCLA2.
GenomeRNAii8803.
PROiQ9P2R7.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000136143.
CleanExiHS_SUCLA2.
ExpressionAtlasiQ9P2R7. baseline and differential.
GenevisibleiQ9P2R7. HS.

Family and domain databases

Gene3Di3.30.1490.20. 1 hit.
3.30.470.20. 1 hit.
3.40.50.261. 1 hit.
HAMAPiMF_00558. Succ_CoA_beta. 1 hit.
MF_03220. Succ_CoA_betaA_euk. 1 hit.
InterProiIPR011761. ATP-grasp.
IPR013650. ATP-grasp_succ-CoA_synth-type.
IPR013815. ATP_grasp_subdomain_1.
IPR013816. ATP_grasp_subdomain_2.
IPR005811. CoA_ligase.
IPR017866. Succ-CoA_synthase_bsu_CS.
IPR005809. Succ_CoA_synthase_bsu.
IPR016102. Succinyl-CoA_synth-like.
[Graphical view]
PANTHERiPTHR11815. PTHR11815. 1 hit.
PfamiPF08442. ATP-grasp_2. 1 hit.
PF00549. Ligase_CoA. 1 hit.
[Graphical view]
PIRSFiPIRSF001554. SucCS_beta. 1 hit.
SUPFAMiSSF52210. SSF52210. 1 hit.
TIGRFAMsiTIGR01016. sucCoAbeta. 1 hit.
PROSITEiPS50975. ATP_GRASP. 1 hit.
PS01217. SUCCINYL_COA_LIG_3. 1 hit.
[Graphical view]
ProtoNetiSearch...

Entry informationi

Entry nameiSUCB1_HUMAN
AccessioniPrimary (citable) accession number: Q9P2R7
Secondary accession number(s): B2RDE7
, O95194, Q5T9Q4, Q5T9Q6, Q9NV21, Q9NVP7
Entry historyi
Integrated into UniProtKB/Swiss-Prot: May 27, 2002
Last sequence update: May 2, 2006
Last modified: November 30, 2016
This is version 172 of the entry and version 3 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

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. Human chromosome 13
    Human chromosome 13: entries, gene names and cross-references to MIM
  2. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  3. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  4. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  5. PATHWAY comments
    Index of metabolic and biosynthesis pathways
  6. 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.