Free for academic non-profit institutions. Other users need a Commercial license

Aliases for SUCLG1 Gene

Aliases for SUCLG1 Gene

  • Succinate-CoA Ligase Alpha Subunit 2
  • Succinate-CoA Ligase, Alpha Subunit 2 3 5
  • Succinyl-CoA Synthetase Subunit Alpha 3 4
  • EC 6.2.1.4 4 63
  • SCS-Alpha 3 4
  • Succinyl-CoA Ligase [GDP-Forming] Subunit Alpha, Mitochondrial 3
  • Succinate-CoA Ligase, GDP-Forming, Alpha Subunit 2
  • EC 6.2.1.5 4
  • EC 6.2.1 63
  • GALPHA 3
  • MTDPS9 3
  • SUCLA1 3

External Ids for SUCLG1 Gene

Previous GeneCards Identifiers for SUCLG1 Gene

  • GC02M084632
  • GC02M084944
  • GC02M084625
  • GC02M084562

Summaries for SUCLG1 Gene

Entrez Gene Summary for SUCLG1 Gene

  • This gene encodes the alpha subunit of the heterodimeric enzyme succinate coenzyme A ligase. This enzyme is targeted to the mitochondria and catalyzes the conversion of succinyl CoA and ADP or GDP to succinate and ATP or GTP. Mutations in this gene are the cause of the metabolic disorder fatal infantile lactic acidosis and mitochondrial DNA depletion. [provided by RefSeq, Feb 2010]

GeneCards Summary for SUCLG1 Gene

SUCLG1 (Succinate-CoA Ligase Alpha Subunit) is a Protein Coding gene. Diseases associated with SUCLG1 include mitochondrial dna depletion syndrome 9 and fatal infantile lactic acidosis with methylmalonic aciduria. Among its related pathways are Carbon metabolism and Citrate cycle (TCA cycle). GO annotations related to this gene include poly(A) RNA binding and GTP binding. An important paralog of this gene is ACLY.

UniProtKB/Swiss-Prot for SUCLG1 Gene

  • Catalyzes the ATP- or GTP-dependent ligation of succinate and CoA to form succinyl-CoA. The nature of the beta subunit determines the nucleotide specificity (By similarity).

Gene Wiki entry for SUCLG1 Gene

No data available for Tocris Summary , PharmGKB "VIP" Summary , fRNAdb sequence ontologies and piRNA Summary for SUCLG1 Gene

Genomics for SUCLG1 Gene

Regulatory Elements for SUCLG1 Gene

Enhancers for SUCLG1 Gene
GeneHancer Identifier Score Enhancer Sources TSS distance (kb) Number of Genes Away Size (kb) Transcription Factor Binding Sites within enhancer Other Gene Targets for Enhancer

Enhancers around SUCLG1 on UCSC Golden Path with GeneCards custom track

Promoters for SUCLG1 Gene
Ensembl Regulatory Elements (ENSRs) TSS Distance (bp) Size (bp) Binding Sites for Transcription Factors within promoters

ENSRs around SUCLG1 on UCSC Golden Path with GeneCards custom track

Genomic Location for SUCLG1 Gene

Chromosome:
2
Start:
84,423,523 bp from pter
End:
84,460,045 bp from pter
Size:
36,523 bases
Orientation:
Minus strand

Genomic View for SUCLG1 Gene

Genes around SUCLG1 on UCSC Golden Path with GeneCards custom track

Cytogenetic band:
SUCLG1 Gene in genomic location: bands according to Ensembl, locations according to GeneLoc (and/or Entrez Gene and/or Ensembl if different)
Genomic Location for SUCLG1 Gene
GeneLoc Logo Genomic Neighborhood Exon StructureGene Density

RefSeq DNA sequence for SUCLG1 Gene

Proteins for SUCLG1 Gene

  • Protein details for SUCLG1 Gene (UniProtKB/Swiss-Prot)

    Protein Symbol:
    P53597-SUCA_HUMAN
    Recommended name:
    Succinyl-CoA ligase [ADP/GDP-forming] subunit alpha, mitochondrial
    Protein Accession:
    P53597
    Secondary Accessions:
    • Q9BWB0
    • Q9UNP6

    Protein attributes for SUCLG1 Gene

    Size:
    346 amino acids
    Molecular mass:
    36250 Da
    Quaternary structure:
    • Heterodimer of an alpha and a beta subunit.
    SequenceCaution:
    • Sequence=AAD17940.2; Type=Erroneous initiation; Evidence={ECO:0000305}; Sequence=AAH00504.1; Type=Erroneous initiation; Evidence={ECO:0000305}; Sequence=CAA92426.1; Type=Erroneous initiation; Evidence={ECO:0000305};

neXtProt entry for SUCLG1 Gene

Proteomics data for SUCLG1 Gene at MOPED

Post-translational modifications for SUCLG1 Gene

  • Ubiquitination at Lys 66 and Lys 280
  • Modification sites at PhosphoSitePlus

Other Protein References for SUCLG1 Gene

ENSEMBL proteins:
REFSEQ proteins:

Antibody Products

  • Cell Signaling Technology (CST) Antibodies for SUCLG1 (SUCLG1)

Domains & Families for SUCLG1 Gene

Suggested Antigen Peptide Sequences for SUCLG1 Gene

Graphical View of Domain Structure for InterPro Entry

P53597

UniProtKB/Swiss-Prot:

SUCA_HUMAN :
  • Belongs to the succinate/malate CoA ligase alpha subunit family.
Family:
  • Belongs to the succinate/malate CoA ligase alpha subunit family.
genes like me logo Genes that share domains with SUCLG1: view

No data available for Gene Families for SUCLG1 Gene

Function for SUCLG1 Gene

Molecular function for SUCLG1 Gene

UniProtKB/Swiss-Prot CatalyticActivity:
GTP + succinate + CoA = GDP + phosphate + succinyl-CoA.
UniProtKB/Swiss-Prot CatalyticActivity:
ATP + succinate + CoA = ADP + phosphate + succinyl-CoA.
UniProtKB/Swiss-Prot Function:
Catalyzes the ATP- or GTP-dependent ligation of succinate and CoA to form succinyl-CoA. The nature of the beta subunit determines the nucleotide specificity (By similarity).

Enzyme Numbers (IUBMB) for SUCLG1 Gene

Gene Ontology (GO) - Molecular Function for SUCLG1 Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0004775 succinate-CoA ligase (ADP-forming) activity IBA --
GO:0005525 GTP binding IEA --
genes like me logo Genes that share ontologies with SUCLG1: view
genes like me logo Genes that share phenotypes with SUCLG1: view

Human Phenotype Ontology for SUCLG1 Gene

HPO Id HPO Name Alternative Ids Definition Synonyms

Animal Model Products

  • Taconic Biosciences Mouse Models for SUCLG1

miRNA for SUCLG1 Gene

miRTarBase miRNAs that target SUCLG1

No data available for Animal Models , Transcription Factor Targets and HOMER Transcription for SUCLG1 Gene

Localization for SUCLG1 Gene

Subcellular locations from UniProtKB/Swiss-Prot for SUCLG1 Gene

Mitochondrion.

Subcellular locations from

COMPARTMENTS
Jensen Localization Image for SUCLG1 Gene COMPARTMENTS Subcellular localization image for SUCLG1 gene
Compartment Confidence
extracellular 5
mitochondrion 5
plasma membrane 5
cytosol 2
endoplasmic reticulum 1

Gene Ontology (GO) - Cellular Components for SUCLG1 Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0005739 mitochondrion IEA,IDA --
genes like me logo Genes that share ontologies with SUCLG1: view

Pathways & Interactions for SUCLG1 Gene

genes like me logo Genes that share pathways with SUCLG1: view

UniProtKB/Swiss-Prot P53597-SUCA_HUMAN

  • Pathway: Carbohydrate metabolism; tricarboxylic acid cycle.

Gene Ontology (GO) - Biological Process for SUCLG1 Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0006104 succinyl-CoA metabolic process IBA --
GO:0006105 succinate metabolic process IBA --
genes like me logo Genes that share ontologies with SUCLG1: view

No data available for SIGNOR curated interactions for SUCLG1 Gene

Drugs & Compounds for SUCLG1 Gene

(6) Drugs for SUCLG1 Gene - From: DrugBank and HMDB

Name Status Disease Links Group Role Mechanism of Action Clinical Trials
Succinic acid Approved Nutra Full agonist, Agonist, Target 0
Adenosine triphosphate Approved Nutra 0
Guanosine diphosphate Experimental Pharma 0
Guanosine triphosphate Experimental Pharma 0
Succinyl-CoA Experimental Pharma 0

(7) Additional Compounds for SUCLG1 Gene - From: HMDB

Name Synonyms Role CAS Number PubChem IDs PubMed IDs
ADP
  • Adenosindiphosphorsaeure
  • Adenosine 5'-pyrophosphate
  • Adenosine diphosphate
  • Adenosine pyrophosphate
  • Adenosine-5'-diphosphate
Full agonist, Agonist 58-64-0
IDP
  • 5'-IDP
  • IDP
  • Inosine 5'-(trihydrogen diphosphate)
  • Inosine 5'-(trihydrogen pyrophosphate)
  • Inosine 5'-diphosphate
86-04-4
inosine triphosphate
  • 2'-Inosine-5'-triphosphate
  • 5'-ITP
  • 5-ITP
  • Inosine 5
  • Inosine 5'-(tetrahydrogen triphosphate)
132-06-9
Itaconic acid
  • Methylenebutanedioate
  • 2-Hydroxy-3-Naphthoyl-2-Naphthylamine
  • 2-Methylenesuccinate
  • 2-Methylenesuccinic acid
  • 2-Propene-1,2-dicarboxylate
97-65-4
Itaconyl-CoA
  • Itaconoyl CoA
  • Itaconoyl coenzyme A
  • S-(1-Hydrogen methylenesuccinate) CoA
  • S-(1-Hydrogen methylenesuccinate) Coenzyme A
  • S-3-Methylenesuccinate CoA
6008-93-1
genes like me logo Genes that share compounds with SUCLG1: view

Transcripts for SUCLG1 Gene

Unigene Clusters for SUCLG1 Gene

Succinate-CoA ligase, alpha subunit:
Representative Sequences:

Alternative Splicing Database (ASD) splice patterns (SP) for SUCLG1 Gene

ExUns: 1 ^ 2a · 2b · 2c ^ 3 ^ 4 ^ 5a · 5b ^ 6 ^ 7a · 7b · 7c ^ 8a · 8b · 8c ^ 9a · 9b ^ 10a · 10b ^ 11
SP1: - - - - -
SP2: -
SP3: - -
SP4: -
SP5: - - - -
SP6: -
SP7: -

Relevant External Links for SUCLG1 Gene

GeneLoc Exon Structure for
SUCLG1
ECgene alternative splicing isoforms for
SUCLG1

Expression for SUCLG1 Gene

mRNA expression in normal human tissues for SUCLG1 Gene

mRNA expression in embryonic tissues and stem cells from LifeMap Discovery

mRNA differential expression in normal tissues according to GTEx for SUCLG1 Gene

This gene is overexpressed in Kidney - Cortex (x4.2).

Protein differential expression in normal tissues from HIPED for SUCLG1 Gene

This gene is overexpressed in Heart (6.1).

Integrated Proteomics: protein expression in normal tissues and cell lines from ProteomicsDB, PaxDb, MOPED, and MaxQB for SUCLG1 Gene



SOURCE GeneReport for Unigene cluster for SUCLG1 Gene Hs.270428

genes like me logo Genes that share expression patterns with SUCLG1: view

Protein tissue co-expression partners for SUCLG1 Gene

- Elite partner

Primer Products

No data available for mRNA Expression by UniProt/SwissProt for SUCLG1 Gene

Orthologs for SUCLG1 Gene

This gene was present in the common ancestor of eukaryotes.

Orthologs for SUCLG1 Gene

Organism Taxonomy Gene Similarity Type Details
cow
(Bos Taurus)
Mammalia SUCLG1 35
  • 89.22 (n)
  • 94.26 (a)
SUCLG1 36
  • 93 (a)
OneToOne
dog
(Canis familiaris)
Mammalia SUCLG1 35
  • 91.54 (n)
  • 95.17 (a)
SUCLG1 36
  • 94 (a)
OneToOne
mouse
(Mus musculus)
Mammalia Suclg1 35
  • 87.19 (n)
  • 93.09 (a)
Suclg1 16
Suclg1 36
  • 92 (a)
OneToOne
chimpanzee
(Pan troglodytes)
Mammalia SUCLG1 35
  • 99.61 (n)
  • 99.42 (a)
SUCLG1 36
  • 99 (a)
OneToOne
rat
(Rattus norvegicus)
Mammalia Suclg1 35
  • 86.99 (n)
  • 90.99 (a)
oppossum
(Monodelphis domestica)
Mammalia SUCLG1 36
  • 77 (a)
OneToOne
platypus
(Ornithorhynchus anatinus)
Mammalia SUCLG1 36
  • 86 (a)
OneToOne
chicken
(Gallus gallus)
Aves SUCLG1 35
  • 81.23 (n)
  • 87.08 (a)
SUCLG1 36
  • 83 (a)
OneToOne
lizard
(Anolis carolinensis)
Reptilia SUCLG1 36
  • 81 (a)
OneToOne
tropical clawed frog
(Silurana tropicalis)
Amphibia Str.7116 35
suclg1 35
  • 75.91 (n)
  • 87.54 (a)
zebrafish
(Danio rerio)
Actinopterygii suclg1 35
  • 73.56 (n)
  • 84.26 (a)
suclg1 36
  • 82 (a)
OneToOne
fruit fly
(Drosophila melanogaster)
Insecta CG6255 37
  • 63 (a)
Scsalpha 37
  • 70 (a)
Scsalpha 35
  • 64.73 (n)
  • 70.32 (a)
CG6255 36
  • 56 (a)
OneToMany
Scsalpha 36
  • 67 (a)
OneToMany
African malaria mosquito
(Anopheles gambiae)
Insecta AgaP_AGAP001312 35
  • 64 (n)
  • 71.69 (a)
worm
(Caenorhabditis elegans)
Secernentea C05G5.4 37
  • 71 (a)
F23H11.3 37
  • 73 (a)
sucl-1 35
  • 64.88 (n)
  • 71.81 (a)
sucl-1 36
  • 66 (a)
OneToMany
sucl-2 36
  • 69 (a)
OneToMany
A. gosspyii yeast
(Ashbya gossypii)
Saccharomycetes AGOS_AFR134C 35
  • 57.97 (n)
  • 60.2 (a)
K. lactis yeast
(Kluyveromyces lactis)
Saccharomycetes KLLA0E03235g 35
  • 59.28 (n)
  • 59.4 (a)
baker's yeast
(Saccharomyces cerevisiae)
Saccharomycetes LSC1 35
  • 61.26 (n)
  • 60.26 (a)
LSC1 36
  • 56 (a)
OneToMany
thale cress
(Arabidopsis thaliana)
eudicotyledons AT5G08300 35
  • 66.44 (n)
  • 71.77 (a)
Alicante grape
(Vitis vinifera)
eudicotyledons Vvi.7530 35
barley
(Hordeum vulgare)
Liliopsida Hv.319 35
rice
(Oryza sativa)
Liliopsida Os.38799 35
wheat
(Triticum aestivum)
Liliopsida Ta.5528 35
corn
(Zea mays)
Liliopsida Zm.6083 35
sea squirt
(Ciona intestinalis)
Ascidiacea Cin.2200 35
bread mold
(Neurospora crassa)
Ascomycetes NCU01227 35
  • 63.16 (n)
  • 69.41 (a)
fission yeast
(Schizosaccharomyces pombe)
Schizosaccharomycetes SPAC16E8.17c 35
  • 61.48 (n)
  • 65.89 (a)
sea squirt
(Ciona savignyi)
Ascidiacea -- 36
  • 71 (a)
OneToOne
Species with no ortholog for SUCLG1:
  • Actinobacteria (Mycobacterium tuberculosis)
  • African clawed frog (Xenopus laevis)
  • alpha proteobacteria (Wolbachia pipientis)
  • amoeba (Dictyostelium discoideum)
  • Archea (Pyrococcus horikoshii)
  • beta proteobacteria (Neisseria meningitidis)
  • Chromalveolata (Phytophthora infestans)
  • common water flea (Daphnia pulex)
  • E. coli (Escherichia coli)
  • filamentous fungi (Aspergillus nidulans)
  • Firmicute bacteria (Streptococcus pneumoniae)
  • green algae (Chlamydomonas reinhardtii)
  • honey bee (Apis mellifera)
  • loblloly pine (Pinus taeda)
  • malaria parasite (Plasmodium falciparum)
  • medicago trunc (Medicago Truncatula)
  • moss (Physcomitrella patens)
  • orangutan (Pongo pygmaeus)
  • pig (Sus scrofa)
  • rainbow trout (Oncorhynchus mykiss)
  • rice blast fungus (Magnaporthe grisea)
  • schistosome parasite (Schistosoma mansoni)
  • sea anemone (Nematostella vectensis)
  • sea urchin (Strongylocentrotus purpuratus)
  • sorghum (Sorghum bicolor)
  • soybean (Glycine max)
  • stem rust fungus (Puccinia graminis)
  • sugarcane (Saccharum officinarum)
  • tomato (Lycopersicon esculentum)
  • toxoplasmosis (Toxoplasma gondii)
  • Trichoplax (Trichoplax adhaerens)

Evolution for SUCLG1 Gene

ENSEMBL:
Gene Tree for SUCLG1 (if available)
TreeFam:
Gene Tree for SUCLG1 (if available)

Paralogs for SUCLG1 Gene

Paralogs for SUCLG1 Gene

genes like me logo Genes that share paralogs with SUCLG1: view

Variants for SUCLG1 Gene

Sequence variations from dbSNP and Humsavar for SUCLG1 Gene

SNP ID Clin Chr 02 pos Sequence Context AA Info Type
VAR_065120 Mitochondrial DNA depletion syndrome 9 (MTDPS9)
VAR_065121 Mitochondrial DNA depletion syndrome 9 (MTDPS9)
VAR_065157 Mitochondrial DNA depletion syndrome 9 (MTDPS9)
rs715788 -- 84,427,928(-) aaaga(A/G)aaaaa intron-variant
rs1599306 -- 84,445,358(+) tggtg(A/G)tgtca intron-variant

Structural Variations from Database of Genomic Variants (DGV) for SUCLG1 Gene

Variant ID Type Subtype PubMed ID
esv2677941 CNV Deletion 23128226

Variation tolerance for SUCLG1 Gene

Residual Variation Intolerance Score: 62.3% of all genes are more intolerant (likely to be disease-causing)
Gene Damage Index Score: 1.16; 23.36% of all genes are more intolerant (likely to be disease-causing)

Relevant External Links for SUCLG1 Gene

HapMap Linkage Disequilibrium report
SUCLG1
Human Gene Mutation Database (HGMD)
SUCLG1

No data available for Polymorphic Variants from UniProtKB/Swiss-Prot for SUCLG1 Gene

Disorders for SUCLG1 Gene

MalaCards: The human disease database

(9) MalaCards diseases for SUCLG1 Gene - From: OMIM, ClinVar, GeneTests, Orphanet, Swiss-Prot, DISEASES, and GeneCards

UniProtKB/Swiss-Prot

SUCA_HUMAN
  • Mitochondrial DNA depletion syndrome 9 (MTDPS9) [MIM:245400]: A 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. {ECO:0000269 PubMed:17668387, ECO:0000269 PubMed:19526370, ECO:0000269 PubMed:20453710, ECO:0000269 PubMed:20693550}. Note=The disease is caused by mutations affecting the gene represented in this entry.

Relevant External Links for SUCLG1

Genetic Association Database (GAD)
SUCLG1
Human Genome Epidemiology (HuGE) Navigator
SUCLG1
Atlas of Genetics and Cytogenetics in Oncology and Haematology:
SUCLG1
genes like me logo Genes that share disorders with SUCLG1: view

No data available for Genatlas for SUCLG1 Gene

Publications for SUCLG1 Gene

  1. The molecular basis for cross-reaction anti-dystrophin antibody with alpha-actinin. (PMID: 9128182) James M. … Morris G.E. (Biochim. Biophys. Acta 1997) 2 3 4 67
  2. Deficiency of the alpha subunit of succinate-coenzyme A ligase causes fatal infantile lactic acidosis with mitochondrial DNA depletion. (PMID: 17668387) Ostergaard E. … Wibrand F. (Am. J. Hum. Genet. 2007) 3 25 26
  3. Marked mitochondrial DNA depletion associated with a novel SUCLG1 gene mutation resulting in lethal neonatal acidosis, multi-organ failure, and interrupted aortic arch. (PMID: 20227526) Rivera H. … Martin M.A. (Mitochondrion 2010) 3 23
  4. The BioPlex Network: A Systematic Exploration of the Human Interactome. (PMID: 26186194) Huttlin E.L. … Gygi S.P. (Cell 2015) 3
  5. Panorama of ancient metazoan macromolecular complexes. (PMID: 26344197) Wan C. … Emili A. (Nature 2015) 3

Products for SUCLG1 Gene

Sources for SUCLG1 Gene

Content