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Gene

SUCLG1

succinate-CoA ligase GDP/ADP-forming subunit alpha

Summary

Cytogenetic location
2p11.2
Coordinates
Chromosome 2: 84,423,528–84,459,280 (GRCh38)
Exons
9 exons across 35.8 kb
Protein
346 amino acids
MANE Select transcript
ENST00000393868.7NM_003849.4
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.

      • 3 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 moderate

How SUCLG1 is laid out

  • Coding sequence
  • Untranslated region
  • Intron
  • Direction of transcription
  • Known disease-causing sites
  • Stretches short reads find hard
Chromosome 2, with a bracket where SUCLG1 sits. SUCLG1 and 3 genes near it on chromosome 2, from 83,941,404 to 84,941,403.Chromosome 22p11.2p25.3p25.2p25.1p24.3p24.2p24.1p23.3p23.2p23.1p22.3p22.2p22.1p21p16.3p16.2p16.1p15p14p13.3p13.2p13.1p12p11.2p11.1q11.1q11.2q12.1q12.2q12.3q13q14.1q14.2q14.3q21.1q21.2q21.3q22.1q22.2q22.3q23.1q23.2q23.3q24.1q24.2q24.3q31.1q31.2q31.3q32.1q32.2q32.3q33.1q33.2q33.3q34q35q36.1q36.2q36.3q37.1q37.2q37.384 Mb84.2 Mb84.4 Mb84.6 Mb84.8 MbSUCLG1: 84,423,528–84,459,280, reverse strandSUCLG1DNAH6: 84,516,455–84,819,589, forward strandDNAH6TRABD2A: 84,821,647–84,907,008, reverse strandTRABD2ATMSB10: 84,904,923–84,906,680, forward strandTMSB10Diagram of SUCLG1: 9 exons on the reverse strand of chromosome 2Exon 8: 2 known disease-causing sitesExon 7: 3 known disease-causing sitesExon 6: 2 known disease-causing sitesExon 5: 1 known disease-causing siteExon 4: 5 known disease-causing sitesExon 3: 1 known disease-causing siteExon 2: 2 known disease-causing sitesExon 1: 2 known disease-causing sitesExon 9: 84,423,528–84,423,772, 245 basesExon 8: 84,425,415–84,425,603, 189 basesExon 7: 84,431,508–84,431,659, 152 basesExon 6: 84,433,352–84,433,435, 84 basesExon 5: 84,441,047–84,441,104, 58 basesExon 4: 84,441,247–84,441,459, 213 basesExon 3: 84,443,284–84,443,400, 117 basesExon 2: 84,449,649–84,449,752, 104 basesExon 1: 84,459,173–84,459,280, 108 bases9864215′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 1.3 kb of the gene's 35.8 kb; 1,041 of those bases code for the protein.

How a genome test reads SUCLG1

23

positions in SUCLG1 where ClinVar classes a change as pathogenic or likely pathogenic

None of them falls in a region short reads find hard.

A whole genome covers all 35.8 kb of SUCLG1, introns included, not only the 1.3 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.). SUCLG1 gene: succinate-CoA ligase GDP/ADP-forming subunit alpha. Retrieved 17 October 2026, from https://dantelabs.com/genes/suclg1

Vancouver

Dante Labs. SUCLG1 gene: succinate-CoA ligase GDP/ADP-forming subunit alpha [Internet]. [cited 17 October 2026]. Available from: https://dantelabs.com/genes/suclg1

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