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Gene

SDHD

succinate dehydrogenase complex subunit D

Summary

Cytogenetic location
11q23.1
Coordinates
Chromosome 11: 112,086,873–112,095,794 (GRCh38)
Exons
4 exons across 8.9 kb
Protein
159 amino acids
Inheritance
Autosomal dominant
Also known as
cybS
Previous symbols
PGL, PGL1
MANE Select transcript
ENST00000375549.8NM_003002.4
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 report relevant to this gene

  • Hereditary Pheochromocytoma and Paraganglioma

    • Carney-Stratakis syndromeAutosomal dominant

      A combination of paraganglioma and gastrointestinal stromal tumour caused by variants in the SDH genes.

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

      Surveillance covers both paragangliomas and gastrointestinal stromal tumours.

    • Hereditary pheochromocytomaAutosomal dominant

      Inherited susceptibility to catecholamine-producing tumours of the adrenal medulla.

      • 1 GenCC submitter rates it definitive or strong
      • On the ACMG secondary findings list
      What knowing changes

      Biochemical screening and imaging allow tumours to be found and removed early.

    • Hereditary pheochromocytoma-paragangliomaAutosomal dominant

      A group of syndromes (PPGL types 1-7, TMEM127- and MAX-related) in which variants in SDHB, SDHD, SDHC, SDHA, SDHAF2, MAX or TMEM127 raise the risk of pheochromocytoma and paraganglioma.

      • ClinGen rates the link definitive
      • 4 GenCC submitters rate it definitive or strong
      • On the ACMG secondary findings list
      What knowing changes

      Regular blood or urine metanephrine testing and periodic whole-body MRI help find tumours early, before they cause blood-pressure crises; relatives can be offered testing.

Inheritance

Autosomal dominant
One changed copy, from either parent, is enough. A parent who has one passes it on to each child with a one-in-two chance.

How SDHD is laid out

  • Coding sequence
  • Untranslated region
  • Intron
  • Direction of transcription
  • Known disease-causing sites
  • Stretches short reads find hard
  • Variants with a page
Chromosome 11, with a bracket where SDHD sits. SDHD and 21 genes near it on chromosome 11, from 111,591,334 to 112,591,333.Chromosome 1111q23.1p15.5p15.4p15.3p15.2p15.1p14.3p14.2p14.1p13p12p11.2p11.12p11.11q11q12.1q12.2q12.3q13.1q13.2q13.3q13.4q13.5q14.1q14.2q14.3q21q22.1q22.2q22.3q23.1q23.2q23.3q24.1q24.2q24.3q25111.6 Mb111.8 Mb112 Mb112.2 Mb112.4 MbSIK2: 111,602,383–111,730,855, forward strandSIK2PPP2R1B: 111,726,908–111,766,713, reverse strandPPP2R1BALG9: 111,782,195–111,872,405, reverse strand: 111,786,286–111,879,425, reverse strandFDXACB1: 111,873,947–111,881,243, reverse strandCFAP68: 111,878,935–111,885,975, forward strandCRYAB: 111,908,239–111,923,722, reverse strandCRYABHSPB2: 111,912,159–111,915,098, forward strandHSPB2-C11orf52: 111,912,736–111,926,871, forward strandHSPB2-C11orf52C11orf52: 111,918,032–111,926,873, forward strandC11orf52DIXDC1: 111,927,144–112,023,091, forward strandDIXDC1DLAT: 112,025,033–112,064,404, forward strandPIH1D2: 112,052,485–112,074,274, reverse strandPIH1D2NKAPD1: 112,074,086–112,085,152, forward strandNKAPD1TIMM8B: 112,082,730–112,086,799, reverse strandTIMM8BSDHD: 112,086,873–112,095,794, forward strandSDHD: 112,086,903–112,193,805, forward strandIL18: 112,142,701–112,164,391, reverse strandIL18TEX12: 112,167,313–112,173,057, forward strandTEX12BCO2: 112,175,472–112,225,895, forward strandBCO2PTS: 112,226,059–112,270,449, forward strandPTSPLET1: 112,248,153–112,260,860, reverse strandPLET1Diagram of SDHD: 4 exons on the forward strand of chromosome 11Exon 1: 13 known disease-causing sitesExon 2: 20 known disease-causing sitesExon 3: 35 known disease-causing sitesExon 4: 29 known disease-causing sitesExon 1: 112,086,873–112,086,959, 87 bases. 1 variant with a page in exon 1. List themExon 2: 112,087,857–112,087,973, 117 bases. 5 variants with a page in exon 2. List themExon 3: 112,088,867–112,089,011, 145 bases. 1 variant with a page in exon 3. List themExon 4: 112,094,805–112,095,794, 990 bases. 4 variants with a page in exon 4. List them12345′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 8.9 kb; 480 of those bases code for the protein.

How a genome test reads SDHD

114

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

50 of them (44%) fall in regions short reads find hard.

  • Low mappability (50 sites)Regions where 100 bp or 250 bp reads cannot be placed uniquely.

A whole genome covers all 8.9 kb of SDHD, introns included, not only the 1.3 kb of exons.

Variants with a page (12)

The SDHD variants with a page here, most review stars first. Each page describes ClinVar's public record of the variant, never a result.

VariantHGVS c.Exon or intronPosition (GRCh38)ClinVar classificationReview status
A33Ifs*35c.94_95delExon 2112,087,895Pathogenic2 stars out of 4 criteria provided, multiple submitters, no conflicts
c.314+3A>CIntron 3112,089,014Likely pathogenic2 stars out of 4 criteria provided, multiple submitters, no conflicts
C11Xc.33C>AExon 1112,086,940Pathogenic2 stars out of 4 criteria provided, multiple submitters, no conflicts
D113Mfs*21c.337_340delExon 4112,094,823Pathogenic2 stars out of 4 criteria provided, multiple submitters, no conflicts
G106Vc.317G>TExon 4112,094,807Pathogenic2 stars out of 4 criteria provided, multiple submitters, no conflicts
L20Cfs*66c.57delExon 2112,087,860Pathogenic2 stars out of 4 criteria provided, multiple submitters, no conflicts
L107Rc.320T>GExon 4112,094,810Pathogenic/likely pathogenic2 stars out of 4 criteria provided, multiple submitters, no conflicts
P81Lc.242C>TExon 3112,088,939Pathogenic2 stars out of 4 criteria provided, multiple submitters, no conflicts
R38Xc.112C>TExon 2112,087,916Pathogenic2 stars out of 4 criteria provided, multiple submitters, no conflicts
S32Xc.95C>AExon 2112,087,899Pathogenic2 stars out of 4 criteria provided, multiple submitters, no conflicts

See all 12 SDHD variants →

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.). SDHD gene: succinate dehydrogenase complex subunit D. Retrieved 16 October 2026, from https://dantelabs.com/genes/sdhd

Vancouver

Dante Labs. SDHD gene: succinate dehydrogenase complex subunit D [Internet]. [cited 16 October 2026]. Available from: https://dantelabs.com/genes/sdhd

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