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Aliases for SDHB Gene

Aliases for SDHB Gene

  • Succinate Dehydrogenase Complex, Subunit B, Iron Sulfur (Ip) 2 3
  • Iron-Sulfur Subunit Of Complex II 3 4
  • SDHIP 3 6
  • SDH2 3 6
  • SDH1 3 4
  • CWS2 3 6
  • PGL4 3 6
  • SDH 3 4
  • IP 3 4
  • Succinate Dehydrogenase [Ubiquinone] Iron-Sulfur Subunit, Mitochondrial 3
  • EC 4

External Ids for SDHB Gene

Previous HGNC Symbols for SDHB Gene

  • SDH1
  • SDH

Previous GeneCards Identifiers for SDHB Gene

  • GC01M017082
  • GC01M016386
  • GC01M016726
  • GC01M017090
  • GC01M017217
  • GC01M017345
  • GC01M015589

Summaries for SDHB Gene

Entrez Gene Summary for SDHB Gene

  • Complex II of the respiratory chain, which is specifically involved in the oxidation of succinate, carries electrons from FADH to CoQ. The complex is composed of four nuclear-encoded subunits and is localized in the mitochondrial inner membrane. The iron-sulfur subunit is highly conserved and contains three cysteine-rich clusters which may comprise the iron-sulfur centers of the enzyme. Sporadic and familial mutations in this gene result in paragangliomas and pheochromocytoma, and support a link between mitochondrial dysfunction and tumorigenesis. [provided by RefSeq, Jul 2008]

GeneCards Summary for SDHB Gene

SDHB (Succinate Dehydrogenase Complex, Subunit B, Iron Sulfur (Ip)) is a Protein Coding gene. Diseases associated with SDHB include paragangliomas 4 and cowden syndrome 2. Among its related pathways are Metabolism and Metabolism. GO annotations related to this gene include electron carrier activity and 2 iron, 2 sulfur cluster binding.

UniProtKB/Swiss-Prot for SDHB Gene

  • Iron-sulfur protein (IP) subunit of succinate dehydrogenase (SDH) that is involved in complex II of the mitochondrial electron transport chain and is responsible for transferring electrons from succinate to ubiquinone (coenzyme Q)

Gene Wiki entry for SDHB Gene

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

Genomics for SDHB Gene

Regulatory Elements for SDHB Gene

Genomic Location for SDHB Gene

17,018,722 bp from pter
17,054,170 bp from pter
35,449 bases
Minus strand

Genomic View for SDHB Gene

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

RefSeq DNA sequence for SDHB Gene

Proteins for SDHB Gene

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

    Protein Symbol:
    Recommended name:
    Succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial
    Protein Accession:
    Secondary Accessions:
    • B2R545
    • Q0QEY7
    • Q9NQ12

    Protein attributes for SDHB Gene

    280 amino acids
    Molecular mass:
    31630 Da
    Name=[2Fe-2S] cluster; Xref=ChEBI:CHEBI:49601; Note=Binds 1 [2Fe-2S] cluster.;
    Name=[3Fe-4S] cluster; Xref=ChEBI:CHEBI:21137; Note=Binds 1 [3Fe-4S] cluster.;
    Name=[4Fe-4S] cluster; Xref=ChEBI:CHEBI:49883; Note=Binds 1 [4Fe-4S] cluster.;
    Quaternary structure:
    • Component of complex II composed of four subunits: the flavoprotein (FP) SDHA, iron-sulfur protein (IP) SDHB, and a cytochrome b560 composed of SDHC and SDHD

neXtProt entry for SDHB Gene

Proteomics data for SDHB Gene at MOPED

Post-translational modifications for SDHB Gene

  • Ubiquitination at Lys233
  • Modification sites at PhosphoSitePlus

Other Protein References for SDHB Gene

ENSEMBL proteins:
Reactome Protein details:
REFSEQ proteins:

Antibody Products

No data available for DME Specific Peptides for SDHB Gene

Domains for SDHB Gene

Gene Families for SDHB Gene

  • comII :Mitochondrial respiratory chain complex / Complex II

Graphical View of Domain Structure for InterPro Entry



  • P21912
  • Contains 1 2Fe-2S ferredoxin-type domain.
  • Contains 1 4Fe-4S ferredoxin-type domain.
  • Belongs to the succinate dehydrogenase/fumarate reductase iron-sulfur protein family.
genes like me logo Genes that share domains with SDHB: view

Function for SDHB Gene

Molecular function for SDHB Gene

GENATLAS Biochemistry:
succinate dehydrogenase 1,iron sulfur protein,inner mitochondrial membrane, component,27.4kDa,of complex II of mitochondrial respiratory chain,oxidative phosphorylation, (OXPHOS),also catalyzing the seventh step of the citric acid cycle
UniProtKB/Swiss-Prot CatalyticActivity:
Succinate + a quinone = fumarate + a quinol
UniProtKB/Swiss-Prot Function:
Iron-sulfur protein (IP) subunit of succinate dehydrogenase (SDH) that is involved in complex II of the mitochondrial electron transport chain and is responsible for transferring electrons from succinate to ubiquinone (coenzyme Q)

Enzyme Numbers (IUBMB) for SDHB Gene

Gene Ontology (GO) - Molecular Function for SDHB Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0005515 protein binding IPI 15961414
GO:0008177 succinate dehydrogenase (ubiquinone) activity IEA --
GO:0009055 electron carrier activity IEA --
GO:0016491 oxidoreductase activity --
GO:0046872 metal ion binding IEA --
genes like me logo Genes that share ontologies with SDHB: view

Phenotypes for SDHB Gene

MGI mutant phenotypes for SDHB:
inferred from 1 alleles
genes like me logo Genes that share phenotypes with SDHB: view

Animal Model Products

miRNA for SDHB Gene

miRTarBase miRNAs that target SDHB

miRNA Products

Inhibitory RNA Products

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

In Situ Assay Products

Flow Cytometry Products

No data available for Animal Models , Transcription Factor Targeting and HOMER Transcription for SDHB Gene

Localization for SDHB Gene

Subcellular locations from UniProtKB/Swiss-Prot for SDHB Gene

Mitochondrion inner membrane; Peripheral membrane protein; Matrix side.

Subcellular locations from

Jensen Localization Image for SDHB Gene COMPARTMENTS Subcellular localization image for SDHB gene
Compartment Confidence
mitochondrion 5
nucleus 2
cytosol 1
extracellular 1
plasma membrane 1

Gene Ontology (GO) - Cellular Components for SDHB Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0005739 mitochondrion IDA --
GO:0005743 mitochondrial inner membrane TAS --
GO:0005749 mitochondrial respiratory chain complex II, succinate dehydrogenase complex (ubiquinone) ISS --
GO:0045273 respiratory chain complex II --
GO:0070062 extracellular exosome IDA 23376485
genes like me logo Genes that share ontologies with SDHB: view

Pathways for SDHB Gene

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

PCR Array Products

  • Pathway & Disease-focused RT² Profiler PCR Arrays

UniProtKB/Swiss-Prot P21912-SDHB_HUMAN

  • Pathway: Carbohydrate metabolism; tricarboxylic acid cycle; fumarate from succinate (eukaryal route): step 1/1

Gene Ontology (GO) - Biological Process for SDHB Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0006099 tricarboxylic acid cycle TAS --
GO:0006105 succinate metabolic process IEA --
GO:0009060 aerobic respiration TAS 2302193
GO:0022904 respiratory electron transport chain TAS --
GO:0044237 cellular metabolic process TAS --
genes like me logo Genes that share ontologies with SDHB: view

Compounds for SDHB Gene

(9) HMDB Compounds for SDHB Gene

Compound Synonyms Cas Number PubMed IDs
  • 1H-Purin-6-amine flavin dinucleotide
Fumaric acid
  • (2E)-But-2-enedioate
  • Armco iron
  • CoQH2
Succinic acid
  • 1,2-Ethanedicarboxylate

(3) Drugbank Compounds for SDHB Gene

Compound Synonyms Cas Number Type Actions PubMed IDs
Succinic acid
  • 1,2-Ethanedicarboxylic acid
110-15-6 target

(64) Novoseek inferred chemical compound relationships for SDHB Gene

Compound -log(P) Hits PubMed IDs
succinate 95.9 122
fumarate 68.1 16
iron-sulfur 64 14
alpha-ketoglutarate 62.2 6
3-nitropropionic acid 61 12
genes like me logo Genes that share compounds with SDHB: view

Transcripts for SDHB Gene

mRNA/cDNA for SDHB Gene

Unigene Clusters for SDHB Gene

Succinate dehydrogenase complex, subunit B, iron sulfur (Ip):
Representative Sequences:

miRNA Products

Inhibitory RNA Products

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

Primer Products

Flow Cytometry Products

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

No ASD Table

Relevant External Links for SDHB Gene

GeneLoc Exon Structure for
ECgene alternative splicing isoforms for

Expression for SDHB Gene

mRNA expression in normal human tissues for SDHB Gene

mRNA expression in embryonic tissues and stem cells from LifeMap Discovery

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

This gene is overexpressed in Heart - Left Ventricle (4.3).

Protein differential expression in normal tissues for SDHB Gene

This gene is overexpressed in Heart (7.1) and Tlymphocyte (6.7).

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

SOURCE GeneReport for Unigene cluster for SDHB Gene Hs.465924

genes like me logo Genes that share expressions with SDHB: view

Expression partners for SDHB Gene

In Situ Assay Products

No data available for mRNA Expression by UniProt/SwissProt for SDHB Gene

Orthologs for SDHB Gene

This gene was present in the common ancestor of eukaryotes.

Orthologs for SDHB Gene

Organism Taxonomy Gene Similarity Type Details
(Bos Taurus)
Mammalia SDHB 35
  • 88.57 (n)
  • 93.93 (a)
  • 94 (a)
(Canis familiaris)
Mammalia SDHB 35
  • 90.48 (n)
  • 95.36 (a)
  • 95 (a)
(Mus musculus)
Mammalia Sdhb 35
  • 85.85 (n)
  • 92.45 (a)
Sdhb 16
Sdhb 36
  • 91 (a)
(Pan troglodytes)
Mammalia SDHB 35
  • 99.52 (n)
  • 100 (a)
  • 100 (a)
(Rattus norvegicus)
Mammalia Sdhb 35
  • 84.65 (n)
  • 91.73 (a)
(Monodelphis domestica)
Mammalia -- 36
  • 86 (a)
-- 36
  • 87 (a)
(Ornithorhynchus anatinus)
Mammalia SDHB 36
  • 62 (a)
(Gallus gallus)
Aves SDHB 35
  • 82.09 (n)
  • 90.19 (a)
-- 36
  • 83 (a)
-- 36
  • 90 (a)
(Anolis carolinensis)
Reptilia SDHB 36
  • 72 (a)
tropical clawed frog
(Silurana tropicalis)
Amphibia sdhb 35
  • 79.98 (n)
  • 90.29 (a)
Str.3423 35
African clawed frog
(Xenopus laevis)
Amphibia sdhb-prov 35
(Danio rerio)
Actinopterygii sdhb 35
  • 76.52 (n)
  • 84.67 (a)
sdhb 36
  • 82 (a)
SDHB (2 of 2) 36
  • 49 (a)
fruit fly
(Drosophila melanogaster)
Insecta CG7349 37
  • 71 (a)
SdhB 37
  • 75 (a)
SdhB 35
  • 65.34 (n)
  • 70.29 (a)
CG7349 36
  • 41 (a)
SdhB 36
  • 64 (a)
African malaria mosquito
(Anopheles gambiae)
Insecta AgaP_AGAP007309 35
  • 64.85 (n)
  • 71.27 (a)
(Caenorhabditis elegans)
Secernentea F42A8.2 37
  • 66 (a)
sdhb-1 35
  • 62.95 (n)
  • 63.94 (a)
sdhb-1 36
  • 58 (a)
A. gosspyii yeast
(Ashbya gossypii)
Saccharomycetes AGOS_ACL065C 35
  • 65.28 (n)
  • 73.44 (a)
K. lactis yeast
(Kluyveromyces lactis)
Saccharomycetes KLLA0E00683g 35
  • 65.43 (n)
  • 72.43 (a)
baker's yeast
(Saccharomyces cerevisiae)
Saccharomycetes SDH2 35
  • 65.73 (n)
  • 72.69 (a)
SDH2 36
  • 68 (a)
SDH2 38
thale cress
(Arabidopsis thaliana)
eudicotyledons SDH2-2 35
  • 62.62 (n)
  • 64.85 (a)
(Glycine max)
eudicotyledons Gma.5536 35
Alicante grape
(Vitis vinifera)
eudicotyledons Vvi.7643 35
(Oryza sativa)
Liliopsida Os.5499 35
Os08g0120000 35
  • 59.75 (n)
  • 60.38 (a)
(Zea mays)
Liliopsida Zm.9421 35
sea squirt
(Ciona intestinalis)
Ascidiacea Cin.4731 35
bread mold
(Neurospora crassa)
Ascomycetes NCU00959 35
  • 61.11 (n)
  • 62.59 (a)
fission yeast
(Schizosaccharomyces pombe)
Schizosaccharomycetes sdh2 35
  • 62.27 (n)
  • 66.4 (a)
sea squirt
(Ciona savignyi)
Ascidiacea CSA.3286 36
  • 64 (a)
Species with no ortholog for SDHB:
  • Actinobacteria (Mycobacterium tuberculosis)
  • 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)
  • stem rust fungus (Puccinia graminis)
  • sugarcane (Saccharum officinarum)
  • tomato (Lycopersicon esculentum)
  • toxoplasmosis (Toxoplasma gondii)
  • Trichoplax (Trichoplax adhaerens)
  • wheat (Triticum aestivum)

Evolution for SDHB Gene

Gene Tree for SDHB (if available)
Gene Tree for SDHB (if available)

Paralogs for SDHB Gene Pseudogenes for SDHB Gene

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

No data available for Paralogs for SDHB Gene

Variants for SDHB Gene

Sequence variations from dbSNP and Humsavar for SDHB Gene

SNP ID Clin Chr 01 pos Sequence Context AA Info Type MAF
rs732679 -- 17,045,190(-) GTATC(A/G)CTCTT intron-variant
rs761420 -- 17,022,352(-) ATGCA(C/G)AGGAA intron-variant
rs910213 -- 17,023,114(-) ACTCA(A/G)AGACA intron-variant
rs978528 -- 17,023,303(-) CTTTT(A/G)CCAGC intron-variant
rs1022580 -- 17,033,181(-) TGAAC(G/T)TTACA intron-variant

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

Variant ID Type Subtype PubMed ID
dgv29e1 CNV Complex 17122850
nsv428421 CNV Gain+Loss 18775914
dgv40e1 CNV Complex 17122850
nsv834269 CNV Loss 17160897
nsv834280 CNV Loss 17160897
esv2662800 CNV Deletion 23128226

Relevant External Links for SDHB Gene

HapMap Linkage Disequilibrium report
Human Gene Mutation Database (HGMD)
Locus Specific Mutation Databases (LSDB)

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

Disorders for SDHB Gene

(5) OMIM Diseases for SDHB Gene (185470)


  • Pheochromocytoma (PCC) [MIM:171300]: A catecholamine-producing tumor of chromaffin tissue of the adrenal medulla or sympathetic paraganglia. The cardinal symptom, reflecting the increased secretion of epinephrine and norepinephrine, is hypertension, which may be persistent or intermittent. {ECO:0000269 PubMed:11404820, ECO:0000269 PubMed:12000816, ECO:0000269 PubMed:12618761, ECO:0000269 PubMed:14500403, ECO:0000269 PubMed:14974914, ECO:0000269 PubMed:15328326, ECO:0000269 PubMed:17634472}. Note=Disease susceptibility is associated with variations affecting the gene represented in this entry.
  • Paragangliomas 4 (PGL4) [MIM:115310]: A neural crest tumor usually derived from the chromoreceptor tissue of a paraganglion. Paragangliomas can develop at various body sites, including the head, neck, thorax and abdomen. Most commonly, they are located in the head and neck region, specifically at the carotid bifurcation, the jugular foramen, the vagal nerve, and in the middle ear. {ECO:0000269 PubMed:11404820, ECO:0000269 PubMed:11897817, ECO:0000269 PubMed:14715873, ECO:0000269 PubMed:14974914, ECO:0000269 PubMed:15328326}. Note=The disease is caused by mutations affecting the gene represented in this entry.
  • Paraganglioma and gastric stromal sarcoma (PGGSS) [MIM:606864]: Gastrointestinal stromal tumors may be sporadic or inherited in an autosomal dominant manner, alone or as a component of a syndrome associated with other tumors, such as in the context of neurofibromatosis type 1 (NF1). Patients have both gastrointestinal stromal tumors and paragangliomas. Susceptibility to the tumors was inherited in an apparently autosomal dominant manner, with incomplete penetrance. {ECO:0000269 PubMed:17804857}. Note=The disease is caused by mutations affecting the gene represented in this entry.
  • Cowden syndrome 2 (CWS2) [MIM:612359]: A form of Cowden syndrome, a hamartomatous polyposis syndrome with age-related penetrance. Cowden syndrome is characterized by hamartomatous lesions affecting derivatives of ectodermal, mesodermal and endodermal layers, macrocephaly, facial trichilemmomas (benign tumors of the hair follicle infundibulum), acral keratoses, papillomatous papules, and elevated risk for development of several types of malignancy, particularly breast carcinoma in women and thyroid carcinoma in both men and women. Colon cancer and renal cell carcinoma have also been reported. Hamartomas can be found in virtually every organ, but most commonly in the skin, gastrointestinal tract, breast and thyroid. {ECO:0000269 PubMed:18678321}. Note=The disease may be caused by mutations affecting the gene represented in this entry.

(55) Novoseek inferred disease relationships for SDHB Gene

Disease -log(P) Hits PubMed IDs
paraganglioma 96.1 186
pheochromocytoma 91 165
paraganglioma extra-adrenal 83 3
multiple endocrine neoplasia 82.7 15
von hippel-lindau disease 82.2 12

Relevant External Links for SDHB

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

No data available for Genatlas for SDHB Gene

Publications for SDHB Gene

  1. Prevalence of SDHB, SDHC, and SDHD germline mutations in clinic patients with head and neck paragangliomas. (PMID: 11897817) Baysal B.E. … Rubinstein W.S. (J. Med. Genet. 2002) 3 4 23 48
  2. Germ-line mutations in nonsyndromic pheochromocytoma. (PMID: 12000816) Neumann H.P.H. … Eng C. (N. Engl. J. Med. 2002) 3 4 23 48
  3. Familial malignant catecholamine-secreting paraganglioma with prolonged survival associated with mutation in the succinate dehydrogenase B gene. (PMID: 12213855) Young A.L. … Young W.F. Jr. (J. Clin. Endocrinol. Metab. 2002) 3 4 23 48
  4. Distinct clinical features of paraganglioma syndromes associated with SDHB and SDHD gene mutations. (PMID: 15328326) Neumann H.P.H. … Eng C. (JAMA 2004) 3 4 23 48
  5. Germline mutations and variants in the succinate dehydrogenase genes in Cowden and Cowden-like syndromes. (PMID: 18678321) Ni Y. … Eng C. (Am. J. Hum. Genet. 2008) 3 4 23 48

Products for SDHB Gene

  • Addgene plasmids for SDHB

Sources for SDHB Gene

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