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Gene

SUCLA2

succinate-CoA ligase ADP-forming subunit beta

Summary

Cytogenetic location
13q14.2
Coordinates
Chromosome 13: 47,942,656–48,001,273 (GRCh38)
Exons
11 exons across 58.6 kb
Protein
463 amino acids
Previous symbols
LINC00444
MANE Select transcript
ENST00000646932.1NM_003850.3
Locus type
gene with protein product
  • Very high
  • Confident
  • Low
  • Very low

Reference genomeGRCh38

Positions on gene and variant pages follow this choice, in this browser.

Curated reports relevant to this gene

  • Primary Mitochondrial Disease

    • Mitochondrial DNA Depletion Syndrome

      Caused by variants in nuclear genes such as POLG, TWNK, TK2, DGUOK, MPV17, RRM2B and SUCLA2, which reduce the amount of mitochondrial DNA and can cause muscle, liver or brain disease.

      • 2 GenCC submitters rate it definitive or strong
      What knowing changes

      Knowing is directly actionable: valproate must be avoided in POLG disease because of the risk of fatal liver failure, and TK2 deficiency has a nucleoside therapy.

Broader groupings

  • Leigh Syndrome

    • One of 69 genes linked to it

      A progressive neurological disease defined by specific neuropathological features associating brainstem and basal ganglia lesions.

      • ClinGen rates the link definitive
      • 1 GenCC submitter rates it definitive or strong

How SUCLA2 is laid out

  • Coding sequence
  • Untranslated region
  • Intron
  • Direction of transcription
  • Known disease-causing sites
  • Stretches short reads find hard
Chromosome 13, with a bracket where SUCLA2 sits. SUCLA2 and 5 genes near it on chromosome 13, from 47,471,965 to 48,471,964.Chromosome 1313q14.2p13p12p11.2p11.1q11q12.11q12.12q12.13q12.2q12.3q13.1q13.2q13.3q14.11q14.12q14.13q14.2q14.3q21.1q21.2q21.31q21.32q21.33q22.1q22.2q22.3q31.1q31.2q31.3q32.1q32.2q32.3q33.1q33.2q33.3q3447.6 Mb47.8 Mb48 Mb48.2 Mb48.4 MbSUCLA2: 47,942,656–48,001,273, reverse strandSUCLA2MED4: 48,001,823–48,095,150, reverse strandMED4NUDT15: 48,037,514–48,054,196, forward strandNUDT15ITM2B: 48,232,612–48,270,357, forward strandITM2BRB1: 48,303,710–48,599,436, forward strandRB1LPAR6: 48,327,364–48,444,704, reverse strandLPAR6Diagram of SUCLA2: 11 exons on the reverse strand of chromosome 13Exon 10: 2 known disease-causing sitesExon 9: 2 known disease-causing sitesExon 8: 3 known disease-causing sitesExon 7: 5 known disease-causing sitesExon 4: 1 known disease-causing siteExon 3: 2 known disease-causing sitesExon 2: 4 known disease-causing sitesExon 1: 2 known disease-causing sitesExon 11: 47,942,656–47,943,445, 790 basesExon 10: 47,948,940–47,949,028, 89 basesExon 9: 47,949,483–47,949,603, 121 basesExon 8: 47,954,140–47,954,282, 143 basesExon 7: 47,954,396–47,954,557, 162 basesExon 6: 47,968,595–47,968,733, 139 basesExon 5: 47,973,264–47,973,392, 129 basesExon 4: 47,988,541–47,988,703, 163 basesExon 3: 47,988,882–47,988,981, 100 basesExon 2: 47,996,843–47,997,023, 181 basesExon 1: 48,001,180–48,001,273, 94 bases1110864215′3′

Exons of the gene's MANE Select transcript (release v1.5), from NCBI and EMBL-EBI, on the GRCh38 reference genome.

Chromosome bands from UCSC's GRCh38 ideogram (2022-10-29).

Nearby genes from Ensembl release 116, on GRCh38.

Exons make up 2.1 kb of the gene's 58.6 kb; 1,392 of those bases code for the protein.

How a genome test reads SUCLA2

27

positions in SUCLA2 where ClinVar classes a change as pathogenic or likely pathogenic

2 of them (7.4%) fall in regions short reads find hard.

  • Low mappability (2 sites)Regions where 100 bp or 250 bp reads cannot be placed uniquely.

A whole genome covers all 58.6 kb of SUCLA2, introns included, not only the 2.1 kb of exons.

Where this comes from

  • HGNC: the gene's symbol, name, other symbols and location.
  • MANE Select v1.5, from NCBI and EMBL-EBI: the transcript, its exons and coding sequence.
  • GenCC and ClinGen, released 2026-10-16: which conditions the gene is linked to, and how strongly.
  • The ACMG secondary findings list, SF v3.2.
  • ClinVar and the GIAB stratifications: known disease-causing sites, and the regions short reads find hard.
  • The Human Phenotype Ontology, release 2026-09-01: the traits linked to the gene. Find out more at hpo.jax.org.
  • Data release 2026.10.16-c6eea6bf.

Cite this page

APA

Dante Labs. (n.d.). SUCLA2 gene: succinate-CoA ligase ADP-forming subunit beta. Retrieved 17 October 2026, from https://dantelabs.com/genes/sucla2

Vancouver

Dante Labs. SUCLA2 gene: succinate-CoA ligase ADP-forming subunit beta [Internet]. [cited 17 October 2026]. Available from: https://dantelabs.com/genes/sucla2

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