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Protein

Succinate--CoA ligase [ADP/GDP-forming] subunit alpha, mitochondrial

Gene

SUCLG1

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

Functioni

Succinyl-CoA synthetase functions in the citric acid cycle (TCA), coupling the hydrolysis of succinyl-CoA to the synthesis of either ATP or GTP and thus represents the only step of substrate-level phosphorylation in the TCA. The alpha subunit of the enzyme binds the substrates coenzyme A and phosphate, while succinate binding and specificity for either ATP or GTP is provided by different beta subunits.UniRule annotation

Catalytic activityi

ATP + succinate + CoA = ADP + phosphate + succinyl-CoA.UniRule annotation
GTP + succinate + CoA = GDP + phosphate + succinyl-CoA.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 annotation
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 [GDP-forming] subunit beta, mitochondrial (SUCLG2), 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/GDP-forming] subunit alpha, mitochondrial (sucD), Succinate--CoA ligase [ADP-forming] subunit beta, mitochondrial (sucC), 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 [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-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 sitei90Coenzyme AUniRule annotation1
Binding sitei207Substrate; shared with subunit betaUniRule annotation1
Active sitei299Tele-phosphohistidine intermediateUniRule annotation1

GO - Molecular functioni

GO - Biological processi

Keywordsi

Molecular functionLigase
Biological processTricarboxylic acid cycle
LigandNucleotide-binding

Enzyme and pathway databases

BioCyciMetaCyc:HS08877-MONOMER
BRENDAi6.2.1.4 2681
6.2.1.5 2681
ReactomeiR-HSA-71403 Citric acid cycle (TCA cycle)
UniPathwayi
UPA00223;UER00999

Names & Taxonomyi

Protein namesi
Recommended name:
Succinate--CoA ligase [ADP/GDP-forming] subunit alpha, mitochondrialUniRule annotation (EC:6.2.1.4UniRule annotation, EC:6.2.1.5UniRule annotation)
Alternative name(s):
Succinyl-CoA synthetase subunit alphaUniRule annotation
Short name:
SCS-alphaUniRule annotation
Gene namesi
Name:SUCLG1UniRule annotation
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 2

Organism-specific databases

EuPathDBiHostDB:ENSG00000163541.11
HGNCiHGNC:11449 SUCLG1
MIMi611224 gene
neXtProtiNX_P53597

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

Involvement in diseasei

Mitochondrial DNA depletion syndrome 9 (MTDPS9)4 Publications
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionA severe disorder due to mitochondrial dysfunction. It is characterized by infantile onset of hypotonia, lactic acidosis, severe psychomotor retardation, progressive neurologic deterioration, and excretion of methylmalonic acid.
See also OMIM:245400
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_06512014M → L in MTDPS9; with progressive liver disease and recurrent hepatic failure. 1 Publication1
Natural variantiVAR_06515785G → A in MTDPS9. 1 PublicationCorresponds to variant dbSNP:rs267607097EnsemblClinVar.1
Natural variantiVAR_065121170P → R in MTDPS9. 1 PublicationCorresponds to variant dbSNP:rs267607099EnsemblClinVar.1

Keywords - Diseasei

Disease mutation, Primary mitochondrial disease

Organism-specific databases

DisGeNETi8802
MalaCardsiSUCLG1
MIMi245400 phenotype
OpenTargetsiENSG00000163541
Orphaneti17 Fatal infantile lactic acidosis with methylmalonic aciduria
PharmGKBiPA36246

Chemistry databases

DrugBankiDB00139 Succinic acid

Polymorphism and mutation databases

BioMutaiSUCLG1
DMDMi223634731

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 40MitochondrionBy similarityAdd BLAST40
ChainiPRO_000003334041 – 346Succinate--CoA ligase [ADP/GDP-forming] subunit alpha, mitochondrialAdd BLAST306

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei54N6-acetyllysineCombined sources1
Modified residuei57N6-acetyllysine; alternateBy similarity1
Modified residuei57N6-succinyllysine; alternateBy similarity1
Modified residuei66N6-acetyllysine; alternateBy similarity1
Modified residuei66N6-succinyllysine; alternateBy similarity1
Modified residuei81N6-acetyllysineBy similarity1
Modified residuei105N6-acetyllysineBy similarity1
Modified residuei338N6-succinyllysineBy similarity1

Keywords - PTMi

Acetylation

Proteomic databases

EPDiP53597
MaxQBiP53597
PaxDbiP53597
PeptideAtlasiP53597
PRIDEiP53597
ProteomicsDBi56587
TopDownProteomicsiP53597

2D gel databases

UCD-2DPAGEiP53597

PTM databases

iPTMnetiP53597
PhosphoSitePlusiP53597

Expressioni

Gene expression databases

BgeeiENSG00000163541 Expressed in 231 organ(s), highest expression level in cortex of kidney
CleanExiHS_SUCLG1
ExpressionAtlasiP53597 baseline and differential
GenevisibleiP53597 HS

Organism-specific databases

HPAiHPA036683
HPA036684

Interactioni

Subunit structurei

Heterodimer of an alpha and a beta subunit. Different beta subunits determine nucleotide specificity. Together with the ATP-specific beta subunit SUCLA2, forms an ADP-forming succinyl-CoA synthetase (A-SCS). Together with the GTP-specific beta subunit SUCLG2 forms a GDP-forming succinyl-CoA synthetase (G-SCS).UniRule annotation

Protein-protein interaction databases

BioGridi114330, 20 interactors
CORUMiP53597
IntActiP53597, 9 interactors
MINTiP53597
STRINGi9606.ENSP00000377446

Structurei

Secondary structure

1346
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details

3D structure databases

ProteinModelPortaliP53597
SMRiP53597
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni64 – 67Coenzyme A bindingUniRule annotation4
Regioni143 – 145Coenzyme A bindingUniRule annotation3

Sequence similaritiesi

Belongs to the succinate/malate CoA ligase alpha subunit family.UniRule annotation

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiKOG1255 Eukaryota
COG0074 LUCA
GeneTreeiENSGT00530000063275
HOGENOMiHOG000239685
HOVERGENiHBG000957
InParanoidiP53597
KOiK01899
OMAiIIFVPPA
OrthoDBiEOG091G0D9C
PhylomeDBiP53597
TreeFamiTF300666

Family and domain databases

Gene3Di3.40.50.261, 1 hit
HAMAPiMF_01988 Succ_CoA_alpha, 1 hit
InterProiView protein in InterPro
IPR017440 Cit_synth/succinyl-CoA_lig_AS
IPR033847 Citrt_syn/SCS-alpha_CS
IPR003781 CoA-bd
IPR005810 CoA_lig_alpha
IPR005811 CoA_ligase
IPR036291 NAD(P)-bd_dom_sf
IPR016102 Succinyl-CoA_synth-like
PfamiView protein in Pfam
PF02629 CoA_binding, 1 hit
PF00549 Ligase_CoA, 1 hit
PIRSFiPIRSF001553 SucCS_alpha, 1 hit
SMARTiView protein in SMART
SM00881 CoA_binding, 1 hit
SUPFAMiSSF51735 SSF51735, 1 hit
SSF52210 SSF52210, 1 hit
TIGRFAMsiTIGR01019 sucCoAalpha, 1 hit
PROSITEiView protein in PROSITE
PS01216 SUCCINYL_COA_LIG_1, 1 hit
PS00399 SUCCINYL_COA_LIG_2, 1 hit

Sequence (1+)i

Sequence statusi: Complete.

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

This entry has 1 described isoform and 1 potential isoform that is computationally mapped.Show allAlign All

P53597-1 [UniParc]FASTAAdd to basket
« Hide
        10         20         30         40         50
MTATLAAAAD IATMVSGSSG LAAARLLSRS FLLPQNGIRH CSYTASRQHL
60 70 80 90 100
YVDKNTKIIC QGFTGKQGTF HSQQALEYGT KLVGGTTPGK GGQTHLGLPV
110 120 130 140 150
FNTVKEAKEQ TGATASVIYV PPPFAAAAIN EAIEAEIPLV VCITEGIPQQ
160 170 180 190 200
DMVRVKHKLL RQEKTRLIGP NCPGVINPGE CKIGIMPGHI HKKGRIGIVS
210 220 230 240 250
RSGTLTYEAV HQTTQVGLGQ SLCVGIGGDP FNGTDFIDCL EIFLNDSATE
260 270 280 290 300
GIILIGEIGG NAEENAAEFL KQHNSGPNSK PVVSFIAGLT APPGRRMGHA
310 320 330 340
GAIIAGGKGG AKEKISALQS AGVVVSMSPA QLGTTIYKEF EKRKML
Length:346
Mass (Da):36,250
Last modified:February 10, 2009 - v4
Checksum:i76EF06F323CD5188
GO

Computationally mapped potential isoform sequencesi

There is 1 potential isoform mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
H7C233H7C233_HUMAN
Succinate--CoA ligase [ADP/GDP-form...
SUCLG1
105Annotation score:

Sequence cautioni

The sequence AAD17940 differs from that shown. Reason: Erroneous initiation. Translation N-terminally extended.Curated
The sequence AAH00504 differs from that shown. Reason: Erroneous initiation. Translation N-terminally extended.Curated
The sequence CAA92426 differs from that shown. Reason: Erroneous initiation. Translation N-terminally extended.Curated

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti19S → N in CAA92426 (PubMed:9128182).Curated1
Sequence conflicti34P → Q in CAA92426 (PubMed:9128182).Curated1
Sequence conflicti39R → Q in CAA92426 (PubMed:9128182).Curated1
Sequence conflicti87T → L in AAD17940 (Ref. 3) Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_06512014M → L in MTDPS9; with progressive liver disease and recurrent hepatic failure. 1 Publication1
Natural variantiVAR_07643237G → A1 PublicationCorresponds to variant dbSNP:rs369610897EnsemblClinVar.1
Natural variantiVAR_06515785G → A in MTDPS9. 1 PublicationCorresponds to variant dbSNP:rs267607097EnsemblClinVar.1
Natural variantiVAR_065121170P → R in MTDPS9. 1 PublicationCorresponds to variant dbSNP:rs267607099EnsemblClinVar.1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AC096770 Genomic DNA No translation available.
BC000504 mRNA Translation: AAH00504.1 Different initiation.
AF104921 mRNA Translation: AAD17940.2 Different initiation.
Z68204 mRNA Translation: CAA92426.1 Different initiation.
CCDSiCCDS1967.2
RefSeqiNP_003840.2, NM_003849.3
UniGeneiHs.270428

Genome annotation databases

EnsembliENST00000393868; ENSP00000377446; ENSG00000163541
GeneIDi8802
KEGGihsa:8802
UCSCiuc002son.4 human

Keywords - Coding sequence diversityi

Polymorphism

Similar proteinsi

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AC096770 Genomic DNA No translation available.
BC000504 mRNA Translation: AAH00504.1 Different initiation.
AF104921 mRNA Translation: AAD17940.2 Different initiation.
Z68204 mRNA Translation: CAA92426.1 Different initiation.
CCDSiCCDS1967.2
RefSeqiNP_003840.2, NM_003849.3
UniGeneiHs.270428

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
6G4QX-ray2.59A42-346[»]
ProteinModelPortaliP53597
SMRiP53597
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi114330, 20 interactors
CORUMiP53597
IntActiP53597, 9 interactors
MINTiP53597
STRINGi9606.ENSP00000377446

Chemistry databases

DrugBankiDB00139 Succinic acid

PTM databases

iPTMnetiP53597
PhosphoSitePlusiP53597

Polymorphism and mutation databases

BioMutaiSUCLG1
DMDMi223634731

2D gel databases

UCD-2DPAGEiP53597

Proteomic databases

EPDiP53597
MaxQBiP53597
PaxDbiP53597
PeptideAtlasiP53597
PRIDEiP53597
ProteomicsDBi56587
TopDownProteomicsiP53597

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000393868; ENSP00000377446; ENSG00000163541
GeneIDi8802
KEGGihsa:8802
UCSCiuc002son.4 human

Organism-specific databases

CTDi8802
DisGeNETi8802
EuPathDBiHostDB:ENSG00000163541.11
GeneCardsiSUCLG1
HGNCiHGNC:11449 SUCLG1
HPAiHPA036683
HPA036684
MalaCardsiSUCLG1
MIMi245400 phenotype
611224 gene
neXtProtiNX_P53597
OpenTargetsiENSG00000163541
Orphaneti17 Fatal infantile lactic acidosis with methylmalonic aciduria
PharmGKBiPA36246
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG1255 Eukaryota
COG0074 LUCA
GeneTreeiENSGT00530000063275
HOGENOMiHOG000239685
HOVERGENiHBG000957
InParanoidiP53597
KOiK01899
OMAiIIFVPPA
OrthoDBiEOG091G0D9C
PhylomeDBiP53597
TreeFamiTF300666

Enzyme and pathway databases

UniPathwayi
UPA00223;UER00999

BioCyciMetaCyc:HS08877-MONOMER
BRENDAi6.2.1.4 2681
6.2.1.5 2681
ReactomeiR-HSA-71403 Citric acid cycle (TCA cycle)

Miscellaneous databases

ChiTaRSiSUCLG1 human
GeneWikiiSUCLG1
GenomeRNAii8802
PROiPR:P53597
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000163541 Expressed in 231 organ(s), highest expression level in cortex of kidney
CleanExiHS_SUCLG1
ExpressionAtlasiP53597 baseline and differential
GenevisibleiP53597 HS

Family and domain databases

Gene3Di3.40.50.261, 1 hit
HAMAPiMF_01988 Succ_CoA_alpha, 1 hit
InterProiView protein in InterPro
IPR017440 Cit_synth/succinyl-CoA_lig_AS
IPR033847 Citrt_syn/SCS-alpha_CS
IPR003781 CoA-bd
IPR005810 CoA_lig_alpha
IPR005811 CoA_ligase
IPR036291 NAD(P)-bd_dom_sf
IPR016102 Succinyl-CoA_synth-like
PfamiView protein in Pfam
PF02629 CoA_binding, 1 hit
PF00549 Ligase_CoA, 1 hit
PIRSFiPIRSF001553 SucCS_alpha, 1 hit
SMARTiView protein in SMART
SM00881 CoA_binding, 1 hit
SUPFAMiSSF51735 SSF51735, 1 hit
SSF52210 SSF52210, 1 hit
TIGRFAMsiTIGR01019 sucCoAalpha, 1 hit
PROSITEiView protein in PROSITE
PS01216 SUCCINYL_COA_LIG_1, 1 hit
PS00399 SUCCINYL_COA_LIG_2, 1 hit
ProtoNetiSearch...

Entry informationi

Entry nameiSUCA_HUMAN
AccessioniPrimary (citable) accession number: P53597
Secondary accession number(s): Q9BWB0, Q9UNP6
Entry historyiIntegrated into UniProtKB/Swiss-Prot: October 1, 1996
Last sequence update: February 10, 2009
Last modified: November 7, 2018
This is version 189 of the entry and version 4 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

3D-structure, Complete proteome, Reference proteome

Documents

  1. SIMILARITY comments
    Index of protein domains and families
  2. Human chromosome 2
    Human chromosome 2: entries, gene names and cross-references to MIM
  3. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  4. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  5. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  6. PATHWAY comments
    Index of metabolic and biosynthesis pathways
  7. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
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Main funding by: National Institutes of Health

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