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Protein

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

Gene

SUCLG1

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score:

Annotation score:5 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the ‘correct annotation’ for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
-Experimental evidence at protein leveli <p>This indicates the type of evidence that supports the existence of the protein. Note that the ‘protein existence’ evidence does not give information on the accuracy or correctness of the sequence(s) displayed.<p><a href='/help/protein_existence' target='_top'>More...</a></p>

<p>This section provides any useful information about the protein, mostly biological knowledge.<p><a href='/help/function_section' target='_top'>More...</a></p>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

<p>This subsection of the <a href="http://www.uniprot.org/help/function_section">Function</a> section describes the catalytic activity of an enzyme, i.e. a chemical reaction that the enzyme catalyzes.<p><a href='/help/catalytic_activity' target='_top'>More...</a></p>Catalytic activityi

<p>This subsection of the <a href="http://www.uniprot.org/help/function_section">'Function'</a> section describes the metabolic pathway(s) associated with a protein.<p><a href='/help/pathway' target='_top'>More...</a></p>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-forming] subunit beta, mitochondrial (SUCLA2), 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 (sucC), Succinate--CoA ligase [ADP-forming] subunit beta, mitochondrial (SUCLA2), Succinate--CoA ligase [ADP-forming] subunit beta, mitochondrial (SUCLA2), Succinate--CoA ligase [ADP/GDP-forming] subunit alpha, mitochondrial (SUCLG1), 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), Succinate--CoA ligase [ADP/GDP-forming] subunit alpha, mitochondrial (sucD), 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/GDP-forming] subunit alpha, mitochondrial (SUCLG1), Succinate--CoA ligase [GDP-forming] subunit beta, mitochondrial (SUCLG2)
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
<p>This subsection of the ‘Function’ section describes the interaction between a single amino acid and another chemical entity. Priority is given to the annotation of physiological ligands.<p><a href='/help/binding' target='_top'>More...</a></p>Binding sitei90Coenzyme AUniRule annotation1
Binding sitei207Substrate; shared with subunit betaUniRule annotation1
<p>This subsection of the ‘Function’ section is used for enzymes and indicates the residues directly involved in catalysis.<p><a href='/help/act_site' target='_top'>More...</a></p>Active sitei299Tele-phosphohistidine intermediateUniRule annotation1

<p>The <a href="http://www.geneontology.org/">Gene Ontology (GO)</a> project provides a set of hierarchical controlled vocabulary split into 3 categories:<p><a href='/help/gene_ontology' target='_top'>More...</a></p>GO - Molecular functioni

GO - Biological processi

<p>UniProtKB Keywords constitute a <a href="http://www.uniprot.org/keywords">controlled vocabulary</a> with a hierarchical structure. Keywords summarise the content of a UniProtKB entry and facilitate the search for proteins of interest.<p><a href='/help/keywords' target='_top'>More...</a></p>Keywordsi

Molecular functionLigase
Biological processTricarboxylic acid cycle
LigandNucleotide-binding

Enzyme and pathway databases

BioCyc Collection of Pathway/Genome Databases

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BioCyci
MetaCyc:HS08877-MONOMER

BRENDA Comprehensive Enzyme Information System

More...
BRENDAi
6.2.1.4 2681
6.2.1.5 2681

Reactome - a knowledgebase of biological pathways and processes

More...
Reactomei
R-HSA-71403 Citric acid cycle (TCA cycle)

UniPathway: a resource for the exploration and annotation of metabolic pathways

More...
UniPathwayi
UPA00223;UER00999

<p>This section provides information about the protein and gene name(s) and synonym(s) and about the organism that is the source of the protein sequence.<p><a href='/help/names_and_taxonomy_section' target='_top'>More...</a></p>Names & Taxonomyi

<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section provides an exhaustive list of all names of the protein, from commonly used to obsolete, to allow unambiguous identification of a protein.<p><a href='/help/protein_names' target='_top'>More...</a></p>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
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section indicates the name(s) of the gene(s) that code for the protein sequence(s) described in the entry. Four distinct tokens exist: ‘Name’, ‘Synonyms’, ‘Ordered locus names’ and ‘ORF names’.<p><a href='/help/gene_name' target='_top'>More...</a></p>Gene namesi
Name:SUCLG1UniRule annotation
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section provides information on the name(s) of the organism that is the source of the protein sequence.<p><a href='/help/organism-name' target='_top'>More...</a></p>OrganismiHomo sapiens (Human)
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section shows the unique identifier assigned by the NCBI to the source organism of the protein. This is known as the ‘taxonomic identifier’ or ‘taxid’.<p><a href='/help/taxonomic_identifier' target='_top'>More...</a></p>Taxonomic identifieri9606 [NCBI]
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section contains the taxonomic hierarchical classification lineage of the source organism. It lists the nodes as they appear top-down in the taxonomic tree, with the more general grouping listed first.<p><a href='/help/taxonomic_lineage' target='_top'>More...</a></p>Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section is present for entries that are part of a <a href="http://www.uniprot.org/proteomes">proteome</a>, i.e. of a set of proteins thought to be expressed by organisms whose genomes have been completely sequenced.<p><a href='/help/proteomes_manual' target='_top'>More...</a></p>Proteomesi
  • UP000005640 <p>A UniProt <a href="http://www.uniprot.org/manual/proteomes_manual">proteome</a> can consist of several components. <br></br>The component name refers to the genomic component encoding a set of proteins.<p><a href='/help/proteome_component' target='_top'>More...</a></p> Componenti: Chromosome 2

Organism-specific databases

Eukaryotic Pathogen Database Resources

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EuPathDBi
HostDB:ENSG00000163541.11

Human Gene Nomenclature Database

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HGNCi
HGNC:11449 SUCLG1

Online Mendelian Inheritance in Man (OMIM)

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MIMi
611224 gene

neXtProt; the human protein knowledge platform

More...
neXtProti
NX_P53597

<p>This section provides information on the location and the topology of the mature protein in the cell.<p><a href='/help/subcellular_location_section' target='_top'>More...</a></p>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

<p>This section provides information on the disease(s) and phenotype(s) associated with a protein.<p><a href='/help/pathology_and_biotech_section' target='_top'>More...</a></p>Pathology & Biotechi

<p>This subsection of the ‘Pathology and Biotech’ section provides information on the disease(s) associated with genetic variations in a given protein. The information is extracted from the scientific literature and diseases that are also described in the <a href="http://www.ncbi.nlm.nih.gov/sites/entrez?db=omim">OMIM</a> database are represented with a <a href="http://www.uniprot.org/diseases">controlled vocabulary</a> in the following way:<p><a href='/help/involvement_in_disease' target='_top'>More...</a></p>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
<p>This subsection of the ‘Sequence’ section describes natural variant(s) of the protein sequence.<p><a href='/help/variant' target='_top'>More...</a></p>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

DisGeNET

More...
DisGeNETi
8802

GeneReviews a resource of expert-authored, peer-reviewed disease descriptions.

More...
GeneReviewsi
SUCLG1

MalaCards human disease database

More...
MalaCardsi
SUCLG1
MIMi245400 phenotype

Open Targets

More...
OpenTargetsi
ENSG00000163541

Orphanet; a database dedicated to information on rare diseases and orphan drugs

More...
Orphaneti
17 Fatal infantile lactic acidosis with methylmalonic aciduria

The Pharmacogenetics and Pharmacogenomics Knowledge Base

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PharmGKBi
PA36246

Chemistry databases

Drug and drug target database

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DrugBanki
DB00139 Succinic acid

Polymorphism and mutation databases

BioMuta curated single-nucleotide variation and disease association database

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BioMutai
SUCLG1

Domain mapping of disease mutations (DMDM)

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DMDMi
223634731

<p>This section describes post-translational modifications (PTMs) and/or processing events.<p><a href='/help/ptm_processing_section' target='_top'>More...</a></p>PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘PTM / Processing’ section describes the extent of a transit peptide.<p><a href='/help/transit' target='_top'>More...</a></p>Transit peptidei1 – 40MitochondrionBy similarityAdd BLAST40
<p>This subsection of the ‘PTM / Processing’ section describes the extent of a polypeptide chain in the mature protein following processing.<p><a href='/help/chain' target='_top'>More...</a></p>ChainiPRO_000003334041 – 346Succinate--CoA ligase [ADP/GDP-forming] subunit alpha, mitochondrialAdd BLAST306

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘PTM / Processing’ section specifies the position and type of each modified residue excluding <a href="http://www.uniprot.org/manual/lipid">lipids</a>, <a href="http://www.uniprot.org/manual/carbohyd">glycans</a> and <a href="http://www.uniprot.org/manual/crosslnk">protein cross-links</a>.<p><a href='/help/mod_res' target='_top'>More...</a></p>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

Encyclopedia of Proteome Dynamics

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EPDi
P53597

MaxQB - The MaxQuant DataBase

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MaxQBi
P53597

PaxDb, a database of protein abundance averages across all three domains of life

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PaxDbi
P53597

PeptideAtlas

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PeptideAtlasi
P53597

PRoteomics IDEntifications database

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PRIDEi
P53597

ProteomicsDB human proteome resource

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ProteomicsDBi
56587

Consortium for Top Down Proteomics

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TopDownProteomicsi
P53597

2D gel databases

University College Dublin 2-DE Proteome Database

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UCD-2DPAGEi
P53597

PTM databases

iPTMnet integrated resource for PTMs in systems biology context

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iPTMneti
P53597

Comprehensive resource for the study of protein post-translational modifications (PTMs) in human, mouse and rat.

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PhosphoSitePlusi
P53597

<p>This section provides information on the expression of a gene at the mRNA or protein level in cells or in tissues of multicellular organisms.<p><a href='/help/expression_section' target='_top'>More...</a></p>Expressioni

Gene expression databases

Bgee dataBase for Gene Expression Evolution

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Bgeei
ENSG00000163541 Expressed in 231 organ(s), highest expression level in cortex of kidney

CleanEx database of gene expression profiles

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CleanExi
HS_SUCLG1

ExpressionAtlas, Differential and Baseline Expression

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ExpressionAtlasi
P53597 baseline and differential

Genevisible search portal to normalized and curated expression data from Genevestigator

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Genevisiblei
P53597 HS

Organism-specific databases

Human Protein Atlas

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HPAi
HPA036683
HPA036684

<p>This section provides information on the quaternary structure of a protein and on interaction(s) with other proteins or protein complexes.<p><a href='/help/interaction_section' target='_top'>More...</a></p>Interactioni

<p>This subsection of the <a href="http://www.uniprot.org/help/interaction_section">'Interaction'</a> section provides information about the protein quaternary structure and interaction(s) with other proteins or protein complexes (with the exception of physiological receptor-ligand interactions which are annotated in the <a href="http://www.uniprot.org/help/function_section">'Function'</a> section).<p><a href='/help/subunit_structure' target='_top'>More...</a></p>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

The Biological General Repository for Interaction Datasets (BioGrid)

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BioGridi
114330, 22 interactors

CORUM comprehensive resource of mammalian protein complexes

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CORUMi
P53597

Protein interaction database and analysis system

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IntActi
P53597, 9 interactors

Molecular INTeraction database

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MINTi
P53597

STRING: functional protein association networks

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STRINGi
9606.ENSP00000377446

<p>This section provides information on the tertiary and secondary structure of a protein.<p><a href='/help/structure_section' target='_top'>More...</a></p>Structurei

Secondary structure

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

3D structure databases

Protein Model Portal of the PSI-Nature Structural Biology Knowledgebase

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ProteinModelPortali
P53597

SWISS-MODEL Repository - a database of annotated 3D protein structure models

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SMRi
P53597

Database of comparative protein structure models

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ModBasei
Search...

MobiDB: a database of protein disorder and mobility annotations

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MobiDBi
Search...

<p>This section provides information on sequence similarities with other proteins and the domain(s) present in a protein.<p><a href='/help/family_and_domains_section' target='_top'>More...</a></p>Family & Domainsi

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘Family and Domains’ section describes a region of interest that cannot be described in other subsections.<p><a href='/help/region' target='_top'>More...</a></p>Regioni64 – 67Coenzyme A bindingUniRule annotation4
Regioni143 – 145Coenzyme A bindingUniRule annotation3

<p>This subsection of the ‘Family and domains’ section provides information about the sequence similarity with other proteins.<p><a href='/help/sequence_similarities' target='_top'>More...</a></p>Sequence similaritiesi

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

Keywords - Domaini

Transit peptide

Phylogenomic databases

evolutionary genealogy of genes: Non-supervised Orthologous Groups

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eggNOGi
KOG1255 Eukaryota
COG0074 LUCA

Ensembl GeneTree

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GeneTreei
ENSGT00940000156351

The HOGENOM Database of Homologous Genes from Fully Sequenced Organisms

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HOGENOMi
HOG000239685

The HOVERGEN Database of Homologous Vertebrate Genes

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HOVERGENi
HBG000957

InParanoid: Eukaryotic Ortholog Groups

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InParanoidi
P53597

KEGG Orthology (KO)

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KOi
K01899

Identification of Orthologs from Complete Genome Data

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OMAi
IIFVPPA

Database of Orthologous Groups

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OrthoDBi
EOG091G0D9C

Database for complete collections of gene phylogenies

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PhylomeDBi
P53597

TreeFam database of animal gene trees

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TreeFami
TF300666

Family and domain databases

Gene3D Structural and Functional Annotation of Protein Families

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Gene3Di
3.40.50.261, 1 hit

HAMAP database of protein families

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HAMAPi
MF_01988 Succ_CoA_alpha, 1 hit

Integrated resource of protein families, domains and functional sites

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InterProi
View 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

Pfam protein domain database

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Pfami
View protein in Pfam
PF02629 CoA_binding, 1 hit
PF00549 Ligase_CoA, 1 hit

PIRSF; a whole-protein classification database

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PIRSFi
PIRSF001553 SucCS_alpha, 1 hit

Simple Modular Architecture Research Tool; a protein domain database

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SMARTi
View protein in SMART
SM00881 CoA_binding, 1 hit

Superfamily database of structural and functional annotation

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SUPFAMi
SSF51735 SSF51735, 1 hit
SSF52210 SSF52210, 1 hit

TIGRFAMs; a protein family database

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TIGRFAMsi
TIGR01019 sucCoAalpha, 1 hit

PROSITE; a protein domain and family database

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PROSITEi
View protein in PROSITE
PS01216 SUCCINYL_COA_LIG_1, 1 hit
PS00399 SUCCINYL_COA_LIG_2, 1 hit

<p>This section displays by default the canonical protein sequence and upon request all isoforms described in the entry. It also includes information pertinent to the sequence(s), including <a href="http://www.uniprot.org/help/sequence_length">length</a> and <a href="http://www.uniprot.org/help/sequences">molecular weight</a>.<p><a href='/help/sequences_section' target='_top'>More...</a></p>Sequence (1+)i

<p>This subsection of the <a href="http://www.uniprot.org/help/sequences_section">Sequence</a> section indicates if the <a href="http://www.uniprot.org/help/canonical_and_isoforms">canonical sequence</a> displayed by default in the entry is complete or not.<p><a href='/help/sequence_status' target='_top'>More...</a></p>Sequence statusi: Complete.

<p>This subsection of the <a href="http://www.uniprot.org/help/sequences_section">Sequence</a> section indicates if the <a href="http://www.uniprot.org/help/canonical_and_isoforms">canonical sequence</a> displayed by default in the entry is in its mature form or if it represents the precursor.<p><a href='/help/sequence_processing' target='_top'>More...</a></p>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
<p>The checksum is a form of redundancy check that is calculated from the sequence. It is useful for tracking sequence updates.</p> <p>It should be noted that while, in theory, two different sequences could have the same checksum value, the likelihood that this would happen is extremely low.</p> <p>However UniProtKB may contain entries with identical sequences in case of multiple genes (paralogs).</p> <p>The checksum is computed as the sequence 64-bit Cyclic Redundancy Check value (CRC64) using the generator polynomial: x<sup>64</sup> + x<sup>4</sup> + x<sup>3</sup> + x + 1. The algorithm is described in the ISO 3309 standard. </p> <p class="publication">Press W.H., Flannery B.P., Teukolsky S.A. and Vetterling W.T.<br /> <strong>Cyclic redundancy and other checksums</strong><br /> <a href="http://www.nrbook.com/b/bookcpdf.php">Numerical recipes in C 2nd ed., pp896-902, Cambridge University Press (1993)</a>)</p> Checksum:i76EF06F323CD5188
GO

<p>In eukaryotic reference proteomes, unreviewed entries that are likely to belong to the same gene are computationally mapped, based on gene identifiers from Ensembl, EnsemblGenomes and model organism databases.<p><a href='/help/gene_centric_isoform_mapping' target='_top'>More...</a></p>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:

Annotation score:1 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the ‘correct annotation’ for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>

<p>This subsection of the ‘Sequence’ section reports difference(s) between the protein sequence shown in the UniProtKB entry and other available protein sequences derived from the same gene.<p><a href='/help/sequence_caution' target='_top'>More...</a></p>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
<p>This subsection of the ‘Sequence’ section reports difference(s) between the canonical sequence (displayed by default in the entry) and the different sequence submissions merged in the entry. These various submissions may originate from different sequencing projects, different types of experiments, or different biological samples. Sequence conflicts are usually of unknown origin.<p><a href='/help/conflict' target='_top'>More...</a></p>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:

EMBL nucleotide sequence database

More...
EMBLi

GenBank nucleotide sequence database

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GenBanki

DNA Data Bank of Japan; a nucleotide sequence database

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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.

The Consensus CDS (CCDS) project

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CCDSi
CCDS1967.2

NCBI Reference Sequences

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RefSeqi
NP_003840.2, NM_003849.3

UniGene gene-oriented nucleotide sequence clusters

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UniGenei
Hs.270428

Genome annotation databases

Ensembl eukaryotic genome annotation project

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Ensembli
ENST00000393868; ENSP00000377446; ENSG00000163541

Database of genes from NCBI RefSeq genomes

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GeneIDi
8802

KEGG: Kyoto Encyclopedia of Genes and Genomes

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KEGGi
hsa:8802

UCSC genome browser

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UCSCi
uc002son.4 human

Keywords - Coding sequence diversityi

Polymorphism

<p>This section provides links to proteins that are similar to the protein sequence(s) described in this entry at different levels of sequence identity thresholds (100%, 90% and 50%) based on their membership in UniProt Reference Clusters (<a href="http://www.uniprot.org/help/uniref">UniRef</a>).<p><a href='/help/similar_proteins_section' target='_top'>More...</a></p>Similar proteinsi

<p>This section is used to point to information related to entries and found in data collections other than UniProtKB.<p><a href='/help/cross_references_section' target='_top'>More...</a></p>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:

Protein Data Bank Europe

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PDBei

Protein Data Bank RCSB

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RCSB PDBi

Protein Data Bank Japan

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PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
6G4QX-ray2.59A42-346[»]
ProteinModelPortaliP53597
SMRiP53597
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi114330, 22 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

Comparative Toxicogenomics Database

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CTDi
8802
DisGeNETi8802
EuPathDBiHostDB:ENSG00000163541.11

GeneCards: human genes, protein and diseases

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GeneCardsi
SUCLG1
GeneReviewsiSUCLG1
HGNCiHGNC:11449 SUCLG1
HPAiHPA036683
HPA036684
MalaCardsiSUCLG1
MIMi245400 phenotype
611224 gene
neXtProtiNX_P53597
OpenTargetsiENSG00000163541
Orphaneti17 Fatal infantile lactic acidosis with methylmalonic aciduria
PharmGKBiPA36246

GenAtlas: human gene database

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GenAtlasi
Search...

Phylogenomic databases

eggNOGiKOG1255 Eukaryota
COG0074 LUCA
GeneTreeiENSGT00940000156351
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

ChiTaRS: a database of human, mouse and fruit fly chimeric transcripts and RNA-sequencing data

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ChiTaRSi
SUCLG1 human

The Gene Wiki collection of pages on human genes and proteins

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GeneWikii
SUCLG1

Database of phenotypes from RNA interference screens in Drosophila and Homo sapiens

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GenomeRNAii
8802

Protein Ontology

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PROi
PR:P53597

The Stanford Online Universal Resource for Clones and ESTs

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SOURCEi
Search...

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

ProtoNet; Automatic hierarchical classification of proteins

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ProtoNeti
Search...

<p>This section provides general information on the entry.<p><a href='/help/entry_information_section' target='_top'>More...</a></p>Entry informationi

<p>This subsection of the ‘Entry information’ section provides a mnemonic identifier for a UniProtKB entry, but it is not a stable identifier. Each reviewed entry is assigned a unique entry name upon integration into UniProtKB/Swiss-Prot.<p><a href='/help/entry_name' target='_top'>More...</a></p>Entry nameiSUCA_HUMAN
<p>This subsection of the ‘Entry information’ section provides one or more accession number(s). These are stable identifiers and should be used to cite UniProtKB entries. Upon integration into UniProtKB, each entry is assigned a unique accession number, which is called ‘Primary (citable) accession number’.<p><a href='/help/accession_numbers' target='_top'>More...</a></p>AccessioniPrimary (citable) accession number: P53597
Secondary accession number(s): Q9BWB0, Q9UNP6
<p>This subsection of the ‘Entry information’ section shows the date of integration of the entry into UniProtKB, the date of the last sequence update and the date of the last annotation modification (‘Last modified’). The version number for both the entry and the <a href="http://www.uniprot.org/help/canonical_and_isoforms">canonical sequence</a> are also displayed.<p><a href='/help/entry_history' target='_top'>More...</a></p>Entry historyiIntegrated into UniProtKB/Swiss-Prot: October 1, 1996
Last sequence update: February 10, 2009
Last modified: December 5, 2018
This is version 190 of the entry and version 4 of the sequence. See complete history.
<p>This subsection of the ‘Entry information’ section indicates whether the entry has been manually annotated and reviewed by UniProtKB curators or not, in other words, if the entry belongs to the Swiss-Prot section of UniProtKB (<strong>reviewed</strong>) or to the computer-annotated TrEMBL section (<strong>unreviewed</strong>).<p><a href='/help/entry_status' target='_top'>More...</a></p>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.

<p>This section contains any relevant information that doesn’t fit in any other defined sections<p><a href='/help/miscellaneous_section' target='_top'>More...</a></p>Miscellaneousi

Keywords - Technical termi

3D-structure, Complete proteome, Reference proteome

Documents

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