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Aliases for SUCLA2 Gene

Aliases for SUCLA2 Gene

  • Succinate-CoA Ligase, ADP-Forming, Beta Subunit 2 3
  • ATP-Specific Succinyl-CoA Synthetase Subunit Beta 3 4
  • Succinyl-CoA Synthetase Beta-A Chain 3 4
  • Renal Carcinoma Antigen NY-REN-39 3 4
  • EC 4 63
  • SCS-BetaA 3 4
  • MTDPS5 3 6
  • Succinyl-CoA Ligase [ADP-Forming] Subunit Beta, Mitochondrial 3
  • Mitochondrial Succinyl-CoA Ligase [ADP-Forming] Subunit Beta 3
  • ATP-Specific Succinyl-CoA Synthetase, Beta Subunit 3
  • EC 6.2.1 63
  • A-BETA 3

External Ids for SUCLA2 Gene

Previous GeneCards Identifiers for SUCLA2 Gene

  • GC13M046516
  • GC13M042504
  • GC13M047215
  • GC13M046314
  • GC13M047414
  • GC13M048510
  • GC13M029309

Summaries for SUCLA2 Gene

Entrez Gene Summary for SUCLA2 Gene

  • Succinyl-CoA synthetase (SCS) is a mitochondrial matrix enzyme that acts as a heterodimer, being composed of an invariant alpha subunit and a substrate-specific beta subunit. The protein encoded by this gene is an ATP-specific SCS beta subunit that dimerizes with the SCS alpha subunit to form SCS-A, an essential component of the tricarboxylic acid cycle. SCS-A hydrolyzes ATP to convert succinate to succinyl-CoA. Defects in this gene are a cause of myopathic mitochondrial DNA depletion syndrome. A pseudogene of this gene has been found on chromosome 6. [provided by RefSeq, Jul 2008]

GeneCards Summary for SUCLA2 Gene

SUCLA2 (Succinate-CoA Ligase, ADP-Forming, Beta Subunit) is a Protein Coding gene. Diseases associated with SUCLA2 include mitochondrial dna depletion syndrome 5 and sucla2-related mitochondrial dna depletion syndrome, encephalomyopathic form, with mild methylmalonic aciduria. Among its related pathways are Metabolism and Metabolism. GO annotations related to this gene include succinate-CoA ligase (ADP-forming) activity. An important paralog of this gene is SUCLG2.

UniProtKB/Swiss-Prot for SUCLA2 Gene

  • Catalyzes the ATP-dependent ligation of succinate and CoA to form succinyl-CoA.

Gene Wiki entry for SUCLA2 Gene

No data available for Tocris Summary , PharmGKB "VIP" Summary , fRNAdb sequence ontologies and piRNA Summary for SUCLA2 Gene

Genomics for SUCLA2 Gene

Regulatory Elements for SUCLA2 Gene

Epigenetics Products

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

Genomic Location for SUCLA2 Gene

47,936,487 bp from pter
48,001,326 bp from pter
64,840 bases
Minus strand

Genomic View for SUCLA2 Gene

UCSC Golden Path with GeneCards custom track
Cytogenetic band:
Genomic Location for SUCLA2 Gene
GeneLoc Logo Genomic Neighborhood Exon StructureGene Density

RefSeq DNA sequence for SUCLA2 Gene

Proteins for SUCLA2 Gene

  • Protein details for SUCLA2 Gene (UniProtKB/Swiss-Prot)

    Protein Symbol:
    Recommended name:
    Succinyl-CoA ligase [ADP-forming] subunit beta, mitochondrial
    Protein Accession:
    Secondary Accessions:
    • B2RDE7
    • O95194
    • Q5T9Q4
    • Q5T9Q6
    • Q9NV21
    • Q9NVP7

    Protein attributes for SUCLA2 Gene

    463 amino acids
    Molecular mass:
    50317 Da
    Quaternary structure:
    • Heterodimer of an alpha and a beta subunit. Interacts with ALAS2.

    Alternative splice isoforms for SUCLA2 Gene


neXtProt entry for SUCLA2 Gene

Proteomics data for SUCLA2 Gene at MOPED

Post-translational modifications for SUCLA2 Gene

  • Ubiquitination at Lys78 and Lys417
  • Modification sites at PhosphoSitePlus

Other Protein References for SUCLA2 Gene

ENSEMBL proteins:
Reactome Protein details:
REFSEQ proteins:

Antibody Products

Domains for SUCLA2 Gene

Graphical View of Domain Structure for InterPro Entry



  • Contains 1 ATP-grasp domain.
  • Belongs to the succinate/malate CoA ligase beta subunit family.
  • Contains 1 ATP-grasp domain.
  • Belongs to the succinate/malate CoA ligase beta subunit family.
genes like me logo Genes that share domains with SUCLA2: view

No data available for Gene Families for SUCLA2 Gene

Function for SUCLA2 Gene

Molecular function for SUCLA2 Gene

UniProtKB/Swiss-Prot CatalyticActivity:
ATP + succinate + CoA = ADP + phosphate + succinyl-CoA
UniProtKB/Swiss-Prot Function:
Catalyzes the ATP-dependent ligation of succinate and CoA to form succinyl-CoA.

Enzyme Numbers (IUBMB) for SUCLA2 Gene

Gene Ontology (GO) - Molecular Function for SUCLA2 Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0003824 catalytic activity --
GO:0004775 succinate-CoA ligase (ADP-forming) activity TAS 10727444
GO:0005515 protein binding IPI 10727444
GO:0005524 ATP binding IEA --
GO:0046872 metal ion binding IEA --
genes like me logo Genes that share ontologies with SUCLA2: view

Animal Model Products

miRNA for SUCLA2 Gene

miRNA Products

Inhibitory RNA Products

  • Predesigned siRNA for gene silencing in human,mouse,rat for SUCLA2

In Situ Assay Products

Flow Cytometry Products

No data available for Phenotypes , Animal Models , Transcription Factor Targets and HOMER Transcription for SUCLA2 Gene

Localization for SUCLA2 Gene

Subcellular locations from UniProtKB/Swiss-Prot for SUCLA2 Gene


Subcellular locations from

Jensen Localization Image for SUCLA2 Gene COMPARTMENTS Subcellular localization image for SUCLA2 gene
Compartment Confidence
mitochondrion 5
cytosol 2
peroxisome 1

Gene Ontology (GO) - Cellular Components for SUCLA2 Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0005739 mitochondrion IDA --
GO:0005759 mitochondrial matrix TAS --
GO:0070062 extracellular exosome IDA 19056867
genes like me logo Genes that share ontologies with SUCLA2: view

Pathways for SUCLA2 Gene

genes like me logo Genes that share pathways with SUCLA2: view

PCR Array Products

  • Pathway & Disease-focused RT² Profiler PCR Arrays

UniProtKB/Swiss-Prot Q9P2R7-SUCB1_HUMAN

  • Pathway: Carbohydrate metabolism; tricarboxylic acid cycle; succinate from succinyl-CoA (ligase route): step 1/1

Gene Ontology (GO) - Biological Process for SUCLA2 Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0006099 tricarboxylic acid cycle TAS --
GO:0006104 succinyl-CoA metabolic process TAS 9765291
GO:0006105 succinate metabolic process IEA --
GO:0006781 succinyl-CoA pathway NAS 10727444
GO:0008152 metabolic process --
genes like me logo Genes that share ontologies with SUCLA2: view

Drugs for SUCLA2 Gene

(1) Drugbank Compounds for SUCLA2 Gene

Compound Status Synonyms Cas Number PubChem ID Type Actions PubMed IDs
Succinic acid
  • 1,2-Ethanedicarboxylic acid
110-15-6 1110 Target approved, nutraceutical

(14) HMDB Compounds for SUCLA2 Gene

Compound Synonyms Cas Number PubMed IDs
Adenosine triphosphate
  • 5'-(Tetrahydrogen triphosphate) Adenosine
  • Adenosindiphosphorsaeure
Coenzyme A
  • Acetoacetyl coenzyme A sodium salt
Guanosine diphosphate
  • 5'-GDP
Guanosine triphosphate
  • 5'-GTP

(1) Novoseek inferred chemical compound relationships for SUCLA2 Gene

Compound -log(P) Hits PubMed IDs
methylmalonic acid 79.4 3
genes like me logo Genes that share compounds with SUCLA2: view

Transcripts for SUCLA2 Gene

Unigene Clusters for SUCLA2 Gene

Succinate-CoA ligase, ADP-forming, beta subunit:
Representative Sequences:

miRNA Products

Inhibitory RNA Products

  • Predesigned siRNA for gene silencing in human,mouse,rat for SUCLA2

Flow Cytometry Products

Alternative Splicing Database (ASD) splice patterns (SP) for SUCLA2 Gene

ExUns: 1a · 1b · 1c · 1d ^ 2 ^ 3 ^ 4 ^ 5a · 5b ^ 6a · 6b ^ 7 ^ 8 ^ 9 ^ 10 ^ 11 ^ 12 ^ 13 ^ 14a · 14b
SP1: - - - -
SP3: -
SP4: - -

Relevant External Links for SUCLA2 Gene

GeneLoc Exon Structure for
ECgene alternative splicing isoforms for

Expression for SUCLA2 Gene

mRNA expression in normal human tissues for SUCLA2 Gene

mRNA expression in embryonic tissues and stem cells from LifeMap Discovery

mRNA differential expression in normal tissues according to GTEx for SUCLA2 Gene

This gene is overexpressed in Muscle - Skeletal (4.1).

Protein differential expression in normal tissues for SUCLA2 Gene

This gene is overexpressed in Heart (10.6).

Integrated Proteomics: protein expression from ProteomicsDB, PaxDb, MOPED, and MaxQB for SUCLA2 Gene

SOURCE GeneReport for Unigene cluster for SUCLA2 Gene Hs.743361

mRNA Expression by UniProt/SwissProt for SUCLA2 Gene

Tissue specificity: Widely expressed. Not expressed in liver and lung.
genes like me logo Genes that share expressions with SUCLA2: view

Expression partners for SUCLA2 Gene

* - Elite partner

Primer Products

In Situ Assay Products

Orthologs for SUCLA2 Gene

This gene was present in the common ancestor of eukaryotes.

Orthologs for SUCLA2 Gene

Organism Taxonomy Gene Similarity Type Details
(Bos Taurus)
Mammalia SUCLA2 35
  • 91.94 (n)
  • 93.3 (a)
  • 94 (a)
(Canis familiaris)
Mammalia SUCLA2 35
  • 93.78 (n)
  • 94.47 (a)
  • 95 (a)
(Mus musculus)
Mammalia Sucla2 35
  • 88.05 (n)
  • 91.14 (a)
Sucla2 16
Sucla2 36
  • 91 (a)
(Pan troglodytes)
Mammalia SUCLA2 35
  • 99.21 (n)
  • 99.78 (a)
  • 100 (a)
(Rattus norvegicus)
Mammalia Sucla2 35
  • 87.26 (n)
  • 89.2 (a)
(Monodelphis domestica)
Mammalia SUCLA2 36
  • 84 (a)
(Ornithorhynchus anatinus)
Mammalia SUCLA2 36
  • 21 (a)
(Gallus gallus)
Aves SUCLA2 35
  • 81.63 (n)
  • 85.68 (a)
  • 85 (a)
(Anolis carolinensis)
Reptilia -- 36
  • 74 (a)
-- 36
  • 71 (a)
-- 36
  • 69 (a)
tropical clawed frog
(Silurana tropicalis)
Amphibia MGC75984 35
sucla2 35
  • 76.25 (n)
  • 81.05 (a)
African clawed frog
(Xenopus laevis)
Amphibia Xl.9091 35
(Danio rerio)
Actinopterygii sucla2 35
  • 71.44 (n)
  • 79.18 (a)
wufj90d07 35
sucla2 36
  • 78 (a)
fruit fly
(Drosophila melanogaster)
Insecta CG11963 37
  • 59 (a)
Sucb 37
  • 49 (a)
skap 35
  • 58.33 (n)
  • 59.47 (a)
skap 36
  • 50 (a)
African malaria mosquito
(Anopheles gambiae)
Insecta AgaP_AGAP004744 35
  • 61.23 (n)
  • 61.8 (a)
(Caenorhabditis elegans)
Secernentea C50F7.4 37
  • 49 (a)
F47B10.1 37
  • 63 (a)
suca-1 35
  • 60.69 (n)
  • 63.2 (a)
suca-1 36
  • 60 (a)
A. gosspyii yeast
(Ashbya gossypii)
Saccharomycetes AGOS_AEL211W 35
  • 49.62 (n)
  • 50.89 (a)
K. lactis yeast
(Kluyveromyces lactis)
Saccharomycetes KLLA0A05478g 35
  • 52.96 (n)
  • 51.78 (a)
baker's yeast
(Saccharomyces cerevisiae)
Saccharomycetes LSC2 35
  • 54.85 (n)
  • 48.61 (a)
LSC2 36
  • 45 (a)
LSC2 38
thale cress
(Arabidopsis thaliana)
eudicotyledons AT2G20420 35
  • 57.35 (n)
  • 52.94 (a)
(Oryza sativa)
Liliopsida Os.9758 35
Os02g0621700 35
  • 57.38 (n)
  • 53.3 (a)
(Zea mays)
Liliopsida Zm.6580 35
sea squirt
(Ciona intestinalis)
Ascidiacea Cin.4817 35
bread mold
(Neurospora crassa)
Ascomycetes NCU08471 35
  • 54.8 (n)
  • 58.6 (a)
fission yeast
(Schizosaccharomyces pombe)
Schizosaccharomycetes SPCC1620.08 35
  • 56.26 (n)
  • 55.21 (a)
sea squirt
(Ciona savignyi)
Ascidiacea CSA.7264 36
  • 70 (a)
Species with no ortholog for SUCLA2:
  • Actinobacteria (Mycobacterium tuberculosis)
  • Alicante grape (Vitis vinifera)
  • alpha proteobacteria (Wolbachia pipientis)
  • amoeba (Dictyostelium discoideum)
  • Archea (Pyrococcus horikoshii)
  • barley (Hordeum vulgare)
  • 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)
  • wheat (Triticum aestivum)

Evolution for SUCLA2 Gene

Gene Tree for SUCLA2 (if available)
Gene Tree for SUCLA2 (if available)

Paralogs for SUCLA2 Gene

Paralogs for SUCLA2 Gene

(4) SIMAP similar genes for SUCLA2 Gene using alignment to 6 proteins:

genes like me logo Genes that share paralogs with SUCLA2: view

Variants for SUCLA2 Gene

Sequence variations from dbSNP and Humsavar for SUCLA2 Gene

SNP ID Clin Chr 13 pos Sequence Context AA Info Type MAF
rs10397 -- 47,942,710(-) AAGGA(A/C/G)TTACA utr-variant-3-prime
rs13243 -- 47,943,043(-) GATAA(A/G)TACGA utr-variant-3-prime
rs15840 -- 47,943,343(-) GAAGG(A/T)GTTAA utr-variant-3-prime
rs718010 -- 47,958,765(-) aataa(G/T)aactt intron-variant
rs765808 -- 47,952,996(+) TGCCT(C/G)TGTCC intron-variant

Structural Variations from Database of Genomic Variants (DGV) for SUCLA2 Gene

Variant ID Type Subtype PubMed ID
nsv1029 CNV Insertion 18451855
nsv832601 CNV Gain 17160897

Relevant External Links for SUCLA2 Gene

HapMap Linkage Disequilibrium report
Human Gene Mutation Database (HGMD)

No data available for Polymorphic Variants from UniProtKB/Swiss-Prot for SUCLA2 Gene

Disorders for SUCLA2 Gene

(1) OMIM Diseases for SUCLA2 Gene (603921)


  • Mitochondrial DNA depletion syndrome 5 (MTDPS5) [MIM:612073]: A disorder due to mitochondrial dysfunction. It is characterized by infantile onset of hypotonia, neurologic deterioration, a hyperkinetic-dystonic movement disorder, external ophthalmoplegia, deafness, variable renal tubular dysfunction, and mild methylmalonic aciduria in some patients. {ECO:0000269 PubMed:15877282, ECO:0000269 PubMed:17287286, ECO:0000269 PubMed:17301081, ECO:0000269 PubMed:23759946}. Note=The disease is caused by mutations affecting the gene represented in this entry.

(2) University of Copenhagen DISEASES for SUCLA2 Gene

Relevant External Links for SUCLA2

Genetic Association Database (GAD)
Human Genome Epidemiology (HuGE) Navigator
genes like me logo Genes that share disorders with SUCLA2: view

No data available for Novoseek inferred disease relationships and Genatlas for SUCLA2 Gene

Publications for SUCLA2 Gene

  1. Interaction between succinyl CoA synthetase and the heme-biosynthetic enzyme ALAS-E is disrupted in sideroblastic anemia. (PMID: 10727444) Furuyama K. … Shigeru S. (J. Clin. Invest. 2000) 3 4 23
  2. Genetic evidence for the expression of ATP- and GTP-specific succinyl-CoA synthetases in multicellular eucaryotes. (PMID: 9765291) Johnson J.D. … Lambeth D.O. (J. Biol. Chem. 1998) 2 3 4
  3. Antigens recognized by autologous antibody in patients with renal- cell carcinoma. (PMID: 10508479) Scanlan M.J. … Old L.J. (Int. J. Cancer 1999) 3 4
  4. The novel mutation p.Asp251Asn in the I^-subunit of succinate-CoA ligase causes encephalomyopathy and elevated succinylcarnitine. (PMID: 23759946) Jaberi E. … Elahi E. (J. Hum. Genet. 2013) 3 4
  5. Mitochondrial encephalomyopathy with elevated methylmalonic acid is caused by SUCLA2 mutations. (PMID: 17287286) Ostergaard E. … Schwartz M. (Brain 2007) 4 23

Products for SUCLA2 Gene

Sources for SUCLA2 Gene

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