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Gene

SDHB

succinate dehydrogenase complex iron sulfur subunit B

Summary

Cytogenetic location
1p36.13
Coordinates
Chromosome 1: 17,018,722–17,054,032 (GRCh38)
Exons
8 exons across 35.3 kb
Protein
280 amino acids
Inheritance
Autosomal dominant
Previous symbols
SDH1, SDH
MANE Select transcript
ENST00000375499.8NM_003000.3
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

  • 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.

  • Kidney Cancer

    • Primary or metastatic malignant neoplasm involving the kidney.

      • 1 GenCC submitter rates it definitive or strong

  • Malignant Endocrine Neoplasm

    • Paragangliomas 4Autosomal dominant

      Any paraganglioma in which the cause of the disease is a mutation in the SDHB gene.

      • 3 GenCC submitters rate it definitive or strong
      • On the ACMG secondary findings list

  • Urinary and Renal System Tumour

    • Urinary System Neoplasm

      A benign or malignant, primary or metastatic neoplasm involving the urinary system.

      • 1 GenCC submitter rates it definitive or strong

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 SDHB 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 1, with a bracket where SDHB sits. SDHB and 11 genes near it on chromosome 1, from 16,536,377 to 17,536,376.Chromosome 11p36.13p36.33p36.32p36.31p36.23p36.22p36.21p36.13p36.12p36.11p35.3p35.2p35.1p34.3p34.2p34.1p33p32.3p32.2p32.1p31.3p31.2p31.1p22.3p22.2p22.1p21.3p21.2p21.1p13.3p13.2p13.1p12p11.2p11.1q11q12q21.1q21.2q21.3q22q23.1q23.2q23.3q24.1q24.2q24.3q25.1q25.2q25.3q31.1q31.2q31.3q32.1q32.2q32.3q41q42.11q42.12q42.13q42.2q42.3q43q4416.6 Mb16.8 Mb17 Mb17.2 Mb17.4 MbNBPF1: 16,562,319–16,613,562, reverse strandNBPF1CROCC: 16,740,273–16,972,964, forward strandCROCCMFAP2: 16,972,933–16,981,616, reverse strandMFAP2ATP13A2: 16,985,958–17,011,986, reverse strandATP13A2: 17,005,068–17,011,757, reverse strandSDHB: 17,018,722–17,054,032, reverse strandSDHBPADI2: 17,066,761–17,119,459, reverse strandPADI2PADI1: 17,205,120–17,246,007, forward strandPADI1PADI3: 17,249,093–17,284,233, forward strandPADI3PADI4: 17,308,195–17,364,004, forward strandPADI4PADI6: 17,372,196–17,401,699, forward strandPADI6RCC2: 17,406,755–17,439,992, reverse strandRCC2Diagram of SDHB: 8 exons on the reverse strand of chromosome 1Exon 8: 10 known disease-causing sitesExon 7: 35 known disease-causing sitesExon 6: 36 known disease-causing sitesExon 5: 26 known disease-causing sitesExon 4: 35 known disease-causing sitesExon 3: 21 known disease-causing sitesExon 2: 25 known disease-causing sitesExon 1: 15 known disease-causing sitesExon 8: 17,018,722–17,018,958, 237 basesExon 7: 17,022,608–17,022,730, 123 bases. 16 variants with a page in exon 7. List themExon 6: 17,023,973–17,024,074, 102 bases. 16 variants with a page in exon 6. List themExon 5: 17,027,749–17,027,865, 117 bases. 2 variants with a page in exon 5. List themExon 4: 17,028,600–17,028,736, 137 bases. 11 variants with a page in exon 4. List themExon 3: 17,033,060–17,033,145, 86 bases. 9 variants with a page in exon 3. List themExon 2: 17,044,761–17,044,888, 128 bases. 11 variants with a page in exon 2. List themExon 1: 17,053,948–17,054,032, 85 bases. 6 variants with a page in exon 1. List them876543215′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 kb of the gene's 35.3 kb; 843 of those bases code for the protein.

How a genome test reads SDHB

235

positions in SDHB 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 35.3 kb of SDHB, introns included, not only the 1 kb of exons.

Variants with a page (87)

The SDHB 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
A15Hfs*4c.42_43insCACTCTCCTTGAGGCGCCGGTTGCCGExon 117,053,977Pathogenic2 stars out of 4 criteria provided, multiple submitters, no conflicts
A15Sfs*3c.19_41dupExon 117,053,978Pathogenic2 stars out of 4 criteria provided, multiple submitters, no conflicts
A25Gfs*38c.71dupExon 117,053,948Pathogenic2 stars out of 4 criteria provided, multiple submitters, no conflicts
c.1A>GExon 117,054,019Pathogenic2 stars out of 4 criteria provided, multiple submitters, no conflicts
c.3G>AExon 117,054,017Pathogenic2 stars out of 4 criteria provided, multiple submitters, no conflicts
c.72+1G>TIntron 117,053,947Pathogenic2 stars out of 4 criteria provided, multiple submitters, no conflicts
c.73-2A>CIntron 117,044,890Likely pathogenic2 stars out of 4 criteria provided, multiple submitters, no conflicts
c.73-2A>TIntron 117,044,890Likely pathogenic2 stars out of 4 criteria provided, multiple submitters, no conflicts
c.200+1G>AIntron 217,044,760Pathogenic2 stars out of 4 criteria provided, multiple submitters, no conflicts
c.201-2A>CIntron 217,033,147Pathogenic2 stars out of 4 criteria provided, multiple submitters, no conflicts

See all 87 SDHB 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.). SDHB gene: succinate dehydrogenase complex iron sulfur subunit B. Retrieved 17 October 2026, from https://dantelabs.com/genes/sdhb

Vancouver

Dante Labs. SDHB gene: succinate dehydrogenase complex iron sulfur subunit B [Internet]. [cited 17 October 2026]. Available from: https://dantelabs.com/genes/sdhb

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