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 3
  • Succinyl-CoA Synthetase Subunit Alpha 3 4
  • EC 4 63
  • SCS-Alpha 3 4
  • MTDPS9 3 6
  • SUCLA1 3 6
  • Succinyl-CoA Ligase [ADP/GDP-Forming] Subunit Alpha, Mitochondrial 3
  • Succinyl-CoA Ligase [GDP-Forming] Subunit Alpha, Mitochondrial 3
  • Succinate-CoA Ligase, GDP-Forming, Alpha Subunit 2
  • EC 4
  • EC 6.2.1 63
  • GALPHA 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. Among its related pathways are Metabolism and Metabolism. GO annotations related to this gene include protein heterodimerization activity and GDP 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

Epigenetics Products

  • DNA Methylation CpG Assay Predesigned for Pyrosequencing in human,mouse,rat

Genomic Location for SUCLG1 Gene

84,423,523 bp from pter
84,460,045 bp from pter
36,523 bases
Minus strand

Genomic View for SUCLG1 Gene

UCSC Golden Path with GeneCards custom track
Cytogenetic band:
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:
    Recommended name:
    Succinyl-CoA ligase [ADP/GDP-forming] subunit alpha, mitochondrial
    Protein Accession:
    Secondary Accessions:
    • Q9BWB0
    • Q9UNP6

    Protein attributes for SUCLG1 Gene

    346 amino acids
    Molecular mass:
    36250 Da
    Quaternary structure:
    • Heterodimer of an alpha and a beta subunit
    • 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 Lys66 and Lys280
  • Modification sites at PhosphoSitePlus

Other Protein References for SUCLG1 Gene

ENSEMBL proteins:
Reactome Protein details:
REFSEQ proteins:

Antibody Products

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

Domains for SUCLG1 Gene

Suggested Antigen Peptide Sequences for SUCLG1 Gene

Graphical View of Domain Structure for InterPro Entry



  • P53597
  • 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:0003824 catalytic activity --
GO:0003878 ATP citrate synthase activity IEA --
GO:0004775 succinate-CoA ligase (ADP-forming) activity IEA --
GO:0004776 succinate-CoA ligase (GDP-forming) activity IEA --
GO:0005525 GTP binding IEA --
genes like me logo Genes that share ontologies with SUCLG1: view

Animal Model Products

CRISPR Products

miRNA for SUCLG1 Gene

miRTarBase miRNAs that target SUCLG1

miRNA Products

Inhibitory RNA Products

  • Predesigned siRNA for gene silencing in human,mouse,rat for SUCLG1

In Situ Assay Products

Flow Cytometry Products

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

Localization for SUCLG1 Gene

Subcellular locations from UniProtKB/Swiss-Prot for SUCLG1 Gene


Subcellular locations from

Jensen Localization Image for SUCLG1 Gene COMPARTMENTS Subcellular localization image for SUCLG1 gene
Compartment Confidence
mitochondrion 5
plasma membrane 4
cytosol 3
endoplasmic reticulum 1
extracellular 1

Gene Ontology (GO) - Cellular Components for SUCLG1 Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0005737 cytoplasm IDA --
GO:0005739 mitochondrion IDA --
GO:0005743 mitochondrial inner membrane IEA --
GO:0005759 mitochondrial matrix TAS --
GO:0005886 plasma membrane IDA --
genes like me logo Genes that share ontologies with SUCLG1: view

Pathways for SUCLG1 Gene

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

PCR Array Products

  • Pathway & Disease-focused RT² Profiler PCR Arrays

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:0006099 tricarboxylic acid cycle TAS --
GO:0006104 succinyl-CoA metabolic process IEA --
GO:0006105 succinate metabolic process IEA --
GO:0008152 metabolic process --
GO:0044237 cellular metabolic process TAS --
genes like me logo Genes that share ontologies with SUCLG1: view

Compounds for SUCLG1 Gene

(10) HMDB Compounds for SUCLG1 Gene

Compound Synonyms Cas Number PubMed IDs
Coenzyme A
  • Acetoacetyl coenzyme A sodium salt
Guanosine diphosphate
  • 5'-GDP
Guanosine triphosphate
  • 5'-GTP
  • 5'-IDP
Inosine triphosphate
  • inosine 5'-(tetrahydrogen triphosphate)

(1) Drugbank Compounds for SUCLG1 Gene

Compound Synonyms Cas Number Type Actions PubMed IDs
Succinic acid
  • 1,2-Ethanedicarboxylic acid
110-15-6 target
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:

CRISPR Products

miRNA Products

Inhibitory RNA Products

  • Predesigned siRNA for gene silencing in human,mouse,rat for SUCLG1

Primer Products

Flow Cytometry Products

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
ECgene alternative splicing isoforms for

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 (4.2).

Integrated Proteomics: protein expression 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 expressions with SUCLG1: view

In Situ Assay 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
(Pan troglodytes)
Mammalia SUCLG1 35
  • 99.61 (n)
  • 99.42 (a)
  • 99 (a)
(Bos Taurus)
Mammalia SUCLG1 35
  • 89.22 (n)
  • 94.26 (a)
  • 93 (a)
(Canis familiaris)
Mammalia SUCLG1 35
  • 91.54 (n)
  • 95.17 (a)
  • 94 (a)
(Mus musculus)
Mammalia Suclg1 35
  • 87.19 (n)
  • 93.09 (a)
Suclg1 16
Suclg1 36
  • 92 (a)
(Monodelphis domestica)
Mammalia SUCLG1 36
  • 77 (a)
(Ornithorhynchus anatinus)
Mammalia SUCLG1 36
  • 86 (a)
(Rattus norvegicus)
Mammalia Suclg1 35
  • 86.99 (n)
  • 90.99 (a)
(Gallus gallus)
Aves SUCLG1 35
  • 81.23 (n)
  • 87.08 (a)
  • 83 (a)
(Anolis carolinensis)
Reptilia SUCLG1 36
  • 81 (a)
tropical clawed frog
(Silurana tropicalis)
Amphibia Str.7116 35
suclg1 35
  • 75.91 (n)
  • 87.54 (a)
(Danio rerio)
Actinopterygii suclg1 35
  • 73.56 (n)
  • 84.26 (a)
suclg1 36
  • 82 (a)
African malaria mosquito
(Anopheles gambiae)
Insecta AgaP_AGAP001312 35
  • 64 (n)
  • 71.69 (a)
fruit fly
(Drosophila melanogaster)
Insecta CG6255 36
  • 56 (a)
Scsalpha 35
  • 64.73 (n)
  • 70.32 (a)
Scsalpha 36
  • 67 (a)
Scsalpha 37
  • 70 (a)
CG6255 37
  • 63 (a)
(Caenorhabditis elegans)
Secernentea sucl-1 35
  • 64.88 (n)
  • 71.81 (a)
sucl-1 36
  • 66 (a)
sucl-2 36
  • 69 (a)
C05G5.4 37
  • 71 (a)
F23H11.3 37
  • 73 (a)
A. gosspyii yeast
(Ashbya gossypii)
Saccharomycetes AGOS_AFR134C 35
  • 57.97 (n)
  • 60.2 (a)
baker's yeast
(Saccharomyces cerevisiae)
Saccharomycetes LSC1 35
  • 61.26 (n)
  • 60.26 (a)
LSC1 36
  • 56 (a)
K. lactis yeast
(Kluyveromyces lactis)
Saccharomycetes KLLA0E03235g 35
  • 59.28 (n)
  • 59.4 (a)
Alicante grape
(Vitis vinifera)
eudicotyledons Vvi.7530 35
thale cress
(Arabidopsis thaliana)
eudicotyledons AT5G08300 35
  • 66.44 (n)
  • 71.77 (a)
(Hordeum vulgare)
Liliopsida Hv.319 35
(Zea mays)
Liliopsida Zm.6083 35
(Oryza sativa)
Liliopsida Os.38799 35
(Triticum aestivum)
Liliopsida Ta.5528 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 intestinalis)
Ascidiacea Cin.2200 35
sea squirt
(Ciona savignyi)
Ascidiacea -- 36
  • 71 (a)
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

Gene Tree for SUCLG1 (if available)
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 MAF
rs2832 -- 84,423,583(+) AAAAC(A/G)TCTTA utr-variant-3-prime
rs715788 -- 84,427,928(-) aaaga(A/G)aaaaa intron-variant
rs957786 -- 84,433,116(+) GCTTC(A/G)GGAGC intron-variant
rs1037857 -- 84,455,618(-) gacat(C/T)gtgat intron-variant
rs1159790 -- 84,458,765(-) GAGGA(A/G)GAGTG intron-variant

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

Variant ID Type Subtype PubMed ID
esv2677941 CNV Deletion 23128226

Relevant External Links for SUCLG1 Gene

HapMap Linkage Disequilibrium report
Human Gene Mutation Database (HGMD)

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

Disorders for SUCLG1 Gene

(1) OMIM Diseases for SUCLG1 Gene (611224)


  • 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.

(3) University of Copenhagen DISEASES for SUCLG1 Gene

Relevant External Links for SUCLG1

Genetic Association Database (GAD)
Human Genome Epidemiology (HuGE) Navigator
genes like me logo Genes that share disorders with SUCLG1: view

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
  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 4 25
  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 status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC). (PMID: 15489334) Gerhard D.S. … Malek J. (Genome Res. 2004) 3 4
  5. A novel missense mutation in SUCLG1 associated with mitochondrial DNA depletion, encephalomyopathic form, with methylmalonic aciduria. (PMID: 19526370) Ostergaard E. … Holme E. (Eur. J. Pediatr. 2010) 3 4

Products for SUCLG1 Gene

Sources for SUCLG1 Gene

Back to Top