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Protein

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

Gene

SUCLG2

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

Functioni

GTP-specific succinyl-CoA synthetase functions in the citric acid cycle (TCA), coupling the hydrolysis of succinyl-CoA to the synthesis of GTP and thus represents the only step of substrate-level phosphorylation in the TCA. 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.

Catalytic activityi

GTP + succinate + CoA = GDP + phosphate + succinyl-CoA.

Cofactori

Mg2+Note: Binds 1 Mg2+ ion per subunit.

Pathwayi: tricarboxylic acid cycle

This protein is involved in step 1 of the subpathway that synthesizes succinate from succinyl-CoA (ligase route).
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 [ADP-forming] subunit beta, mitochondrial (SUCLA2), Succinate--CoA ligase [ADP/GDP-forming] subunit alpha, mitochondrial (SUCLG1), Succinate--CoA ligase [GDP-forming] subunit beta, mitochondrial (SUCLG2), Succinate--CoA ligase [ADP/GDP-forming] subunit alpha, mitochondrial (SUCLG1), Succinate--CoA ligase [ADP-forming] subunit beta, mitochondrial (SUCLA2), Succinate--CoA ligase [ADP/GDP-forming] subunit alpha, mitochondrial (SUCLG1), Succinate--CoA ligase [ADP-forming] subunit beta, mitochondrial (SUCLA2), Succinate--CoA ligase [ADP-forming] subunit beta, mitochondrial (SUCLA2), Succinate--CoA ligase [GDP-forming] subunit beta, mitochondrial (SUCLG2), Succinate--CoA ligase [ADP-forming] subunit beta, mitochondrial (SUCLA2), 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
Binding sitei57GTP1
Sitei79Important for substrate specificity1
Binding sitei146GTP; via amide nitrogen and carbonyl oxygen1
Sitei147Important for substrate specificity1
Metal bindingi243Magnesium1
Metal bindingi257Magnesium1
Binding sitei308Substrate; shared with subunit alpha1

Regions

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Nucleotide bindingi90 – 92GTP3

GO - Molecular functioni

GO - Biological processi

  • succinate metabolic process Source: Ensembl
  • succinyl-CoA metabolic process Source: UniProtKB
  • tricarboxylic acid cycle Source: Reactome

Keywordsi

Molecular functionLigase
Biological processTricarboxylic acid cycle
LigandGTP-binding, Magnesium, Metal-binding, Nucleotide-binding

Enzyme and pathway databases

BioCyciMetaCyc:HS10493-MONOMER.
BRENDAi6.2.1.4. 2681.
ReactomeiR-HSA-71403. Citric acid cycle (TCA cycle).
UniPathwayiUPA00223; UER00999.

Names & Taxonomyi

Protein namesi
Recommended name:
Succinate--CoA ligase [GDP-forming] subunit beta, mitochondrial (EC:6.2.1.4)
Alternative name(s):
GTP-specific succinyl-CoA synthetase subunit beta
Short name:
G-SCS
Short name:
GTPSCS
Succinyl-CoA synthetase beta-G chain
Short name:
SCS-betaG
Gene namesi
Name:SUCLG2
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 3

Organism-specific databases

EuPathDBiHostDB:ENSG00000172340.14.
HGNCiHGNC:11450. SUCLG2.

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

Pathology & Biotechi

Organism-specific databases

DisGeNETi8801.
OpenTargetsiENSG00000172340.
PharmGKBiPA36247.

Chemistry databases

DrugBankiDB00139. Succinic acid.

Polymorphism and mutation databases

BioMutaiSUCLG2.
DMDMi52788292.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 37MitochondrionCombined sourcesAdd BLAST37
ChainiPRO_000003335638 – 432Succinate--CoA ligase [GDP-forming] subunit beta, mitochondrialAdd BLAST395

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei73N6-acetyllysineBy similarity1
Modified residuei78N6-succinyllysineBy similarity1
Modified residuei132N6-acetyllysineBy similarity1
Modified residuei139N6-acetyllysineBy similarity1
Modified residuei161PhosphoserineBy similarity1
Modified residuei200N6-acetyllysineBy similarity1
Modified residuei218N6-acetyllysineBy similarity1
Modified residuei227N6-acetyllysineCombined sources1
Modified residuei271N6-acetyllysineBy similarity1
Modified residuei291N6-acetyllysineCombined sources1
Modified residuei338N6-succinyllysineBy similarity1
Modified residuei347N6-acetyllysineBy similarity1
Modified residuei386N6-acetyllysineBy similarity1
Modified residuei423N6-acetyllysineBy similarity1

Keywords - PTMi

Acetylation, Phosphoprotein

Proteomic databases

EPDiQ96I99.
MaxQBiQ96I99.
PaxDbiQ96I99.
PeptideAtlasiQ96I99.
PRIDEiQ96I99.
TopDownProteomicsiQ96I99-1. [Q96I99-1]
Q96I99-2. [Q96I99-2]

2D gel databases

REPRODUCTION-2DPAGEiIPI00096066.

PTM databases

iPTMnetiQ96I99.
PhosphoSitePlusiQ96I99.
SwissPalmiQ96I99.

Expressioni

Tissue specificityi

Mainly expressed in liver, kidney, heart, spleen and skeletal muscle. Also found in intestine and colon, and in low amounts in lung, brain, prostate, testis and ovary.1 Publication

Gene expression databases

BgeeiENSG00000172340.
CleanExiHS_SUCLG2.
ExpressionAtlasiQ96I99. baseline and differential.
GenevisibleiQ96I99. HS.

Organism-specific databases

HPAiHPA046705.
HPA051998.

Interactioni

Subunit structurei

Heterodimer of an alpha and a beta subunit. The beta subunit determines specificity for GTP.

Binary interactionsi

WithEntry#Exp.IntActNotes
HSCBQ8IWL34EBI-2511878,EBI-1805738

GO - Molecular functioni

Protein-protein interaction databases

BioGridi114329. 42 interactors.
CORUMiQ96I99.
DIPiDIP-53563N.
IntActiQ96I99. 9 interactors.
STRINGi9606.ENSP00000419325.

Structurei

3D structure databases

ProteinModelPortaliQ96I99.
SMRiQ96I99.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini46 – 274ATP-graspAdd BLAST229

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni365 – 367Substrate binding; shared with subunit alpha3

Sequence similaritiesi

Belongs to the succinate/malate CoA ligase beta subunit family. GTP-specific subunit beta subfamily.

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiKOG2799. Eukaryota.
COG0045. LUCA.
GeneTreeiENSGT00390000010170.
HOGENOMiHOG000007059.
HOVERGENiHBG055555.
InParanoidiQ96I99.
KOiK01900.
OMAiVQIEINP.
OrthoDBiEOG091G07T9.
PhylomeDBiQ96I99.
TreeFamiTF300624.

Family and domain databases

Gene3Di3.30.1490.20. 1 hit.
3.40.50.261. 1 hit.
HAMAPiMF_00558. Succ_CoA_beta. 1 hit.
MF_03221. Succ_CoA_betaG_euk. 1 hit.
InterProiView protein in InterPro
IPR013650. ATP-grasp_succ-CoA_synth-type.
IPR013815. ATP_grasp_subdomain_1.
IPR005811. CoA_ligase.
IPR017866. Succ-CoA_synthase_bsu_CS.
IPR034722. Succ_CoA_betaG_euk.
IPR005809. Succ_CoA_synthase_bsu.
IPR016102. Succinyl-CoA_synth-like.
PANTHERiPTHR11815. PTHR11815. 1 hit.
PfamiView protein in Pfam
PF08442. ATP-grasp_2. 1 hit.
PF00549. Ligase_CoA. 1 hit.
PIRSFiPIRSF001554. SucCS_beta. 1 hit.
SUPFAMiSSF52210. SSF52210. 1 hit.
TIGRFAMsiTIGR01016. sucCoAbeta. 1 hit.
PROSITEiView protein in PROSITE
PS01217. SUCCINYL_COA_LIG_3. 1 hit.

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: Q96I99-1) [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
MASPVAAQAG KLLRALALRP RFLAAGSQAV QLTSRRWLNL QEYQSKKLMS
60 70 80 90 100
DNGVRVQRFF VADTANEALE AAKRLNAKEI VLKAQILAGG RGKGVFNSGL
110 120 130 140 150
KGGVHLTKDP NVVGQLAKQM IGYNLATKQT PKEGVKVNKV MVAEALDISR
160 170 180 190 200
ETYLAILMDR SCNGPVLVGS PQGGVDIEEV AASNPELIFK EQIDIFEGIK
210 220 230 240 250
DSQAQRMAEN LGFVGPLKSQ AADQITKLYN LFLKIDATQV EVNPFGETPE
260 270 280 290 300
GQVVCFDAKI NFDDNAEFRQ KDIFAMDDKS ENEPIENEAA KYDLKYIGLD
310 320 330 340 350
GNIACFVNGA GLAMATCDII FLNGGKPANF LDLGGGVKEA QVYQAFKLLT
360 370 380 390 400
ADPKVEAILV NIFGGIVNCA IIANGITKAC RELELKVPLV VRLEGTNVQE
410 420 430
AQKILNNSGL PITSAIDLED AAKKAVASVA KK
Length:432
Mass (Da):46,511
Last modified:September 27, 2004 - v2
Checksum:i56A977A3E50713A1
GO
Isoform 2 (identifier: Q96I99-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     397-432: NVQEAQKILN...KKAVASVAKK → FMEKKGSYMH...SKCAISIFLC

Note: No experimental confirmation available.
Show »
Length:440
Mass (Da):47,732
Checksum:iFFBA486CDA19334B
GO

Sequence cautioni

The sequence AAH07716 differs from that shown. Reason: Erroneous initiation.Curated
The sequence AAH47024 differs from that shown. Reason: Erroneous initiation. Translation N-terminally shortened.Curated
The sequence AAH47024 differs from that shown. Reason: Erroneous termination at position 31. Translated as Gln.Curated

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti174 – 175GV → RS in AAC64397 (PubMed:9765291).Curated2
Sequence conflicti220Q → K in AAH68602 (PubMed:15489334).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_052499347K → R. Corresponds to variant dbSNP:rs9843840Ensembl.1
Natural variantiVAR_052500381R → W. Corresponds to variant dbSNP:rs7623258Ensembl.1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_042013397 – 432NVQEA…SVAKK → FMEKKGSYMHIKQETGNSNE NITGIQENVAHQNLSKCAIS IFLC in isoform 2. 1 PublicationAdd BLAST36

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AK310527 mRNA. No translation available.
AC099783 Genomic DNA. No translation available.
AC112213 Genomic DNA. No translation available.
AC113169 Genomic DNA. No translation available.
AC114401 Genomic DNA. No translation available.
BC007716 mRNA. Translation: AAH07716.3. Different initiation.
BC019868 mRNA. Translation: AAH19868.1.
BC047024 mRNA. Translation: AAH47024.1. Sequence problems.
BC068602 mRNA. Translation: AAH68602.1.
AF058954 mRNA. Translation: AAC64397.1.
AF131748 mRNA. Translation: AAD20032.1.
CCDSiCCDS43104.1. [Q96I99-1]
CCDS54605.1. [Q96I99-2]
RefSeqiNP_001171070.1. NM_001177599.1. [Q96I99-2]
NP_003839.2. NM_003848.3. [Q96I99-1]
UniGeneiHs.644919.

Genome annotation databases

EnsembliENST00000307227; ENSP00000307432; ENSG00000172340. [Q96I99-1]
ENST00000493112; ENSP00000419325; ENSG00000172340. [Q96I99-2]
GeneIDi8801.
KEGGihsa:8801.
UCSCiuc003dna.5. human. [Q96I99-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Similar proteinsi

Entry informationi

Entry nameiSUCB2_HUMAN
AccessioniPrimary (citable) accession number: Q96I99
Secondary accession number(s): C9JVT2
, O95195, Q6NUH7, Q86VX8, Q8WUQ1
Entry historyiIntegrated into UniProtKB/Swiss-Prot: May 27, 2002
Last sequence update: September 27, 2004
Last modified: October 25, 2017
This is version 170 of the entry and version 2 of the sequence. See 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 3
    Human chromosome 3: 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