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FLASH GENE
Symbol SDHB contributors: mct/npt - updated : 08-11-2016
HGNC name succinate dehydrogenase complex, subunit B, iron sulfur (Ip)
HGNC id 10681
Corresponding disease
CWS2 Cowden syndrome 2
PGL4 hereditary paraganglioma 4
SDHBD succinate dehydrogenase subunit B defieciency
Location 1p36.13      Physical location : 17.345.226 - 17.380.665
Synonym name
  • complex 2 of the respiratory chain, succinate-ubiquinone oxidoreductase
  • iron-sulfur subunit of complex II
  • Synonym symbol(s) SDH, IP, SDHIP, PGL4, SDH1, FLJ92337
    EC.number 1.3.5.1, 1.3.99.1
    DNA
    TYPE functioning gene
    STRUCTURE 35.44 kb     8 Exon(s)
    Genomic sequence alignment details
    10 Kb 5' upstream gene genomic sequence study
    MAPPING cloned Y linked N status provisional
    Physical map
    EPHA2 1p36.1 EphA2 FLJ33962 1p36.13 hypothetical protein FLJ33962 LOC343531 1p36.13 similar to oral cancer overexpressed 2; transmembrane protein 16A (eight membrane-spanning domains) MGC10731 1p36.13 hypothetical protein MGC10731 KIAA1332 1p36.23-p36.11 KIAA1332 protein DKFZp566C0424 1p36.13 putative MAPK activating protein PM20,PM21 LOC391008 1 similar to ribosomal protein L22 FLJ10420 1p36.13 hypothetical protein FLJ10420 KIAA1922 1p36.13 KIAA1922 protein LOC391009 1 hypothetical gene supported by AB007923 FLJ20719 1p31 hypothetical protein FLJ20719 LOC388597 1 LOC388597 MGC12760 1p36.13 hypothetical protein MGC12760 LOC388598 1 similar to ring finger protein 123 MSTP2 1p36.2 macrophage stimulating, pseudogene 2 EIF1AP1 1q42.3 eukaryotic translation initiation factor 1A pseudogene 1 LOC284729 1p36.13 hypothetical protein LOC284729 MSTP7 1p36.2 macrophage stimulating, pseudogene 7 LOC391010 1 similar to KIAA0454 protein LOC388599 1 LOC388599 CROCC 1pter-p36.11 ciliary rootlet coiled-coil, rootletin MFAP2 1p36.1 microfibrillar-associated protein 2 HSA9947 1p36 putative ATPase SDHB 1p36.1-p35 succinate dehydrogenase complex, subunit B, iron sulfur (Ip) PADI2 1p35.2-p35.1 peptidyl arginine deiminase, type II PADI1 1p36.13 peptidyl arginine deiminase, type I PADI3 1p36.13 peptidyl arginine deiminase, type III PADI4 1p36.13 peptidyl arginine deiminase, type IV LOC391011 1 similar to Peptidyl arginine deiminase, egg and embryo abundant TD-60 1p36.13 RCC1-like FLJ10521 1p36.13 hypothetical protein FLJ10521 LOC81569 1p36.2-p35 actin like protein
    RNA
    TRANSCRIPTS type messenger
    identificationnb exonstypebpproduct
    ProteinkDaAAspecific expressionYearPubmed
    8 - 1161 28.8 280 - 1995 7622059
    EXPRESSION
    Type ubiquitous
       expressed in (based on citations)
    organ(s)
    SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
    Digestiveliver    
     stomach   highly
    Reproductivefemale systemuteruscervix highly
    Skin/Tegumentskin   highly
    tissue
    SystemTissueTissue level 1Tissue level 2LevelPubmedSpeciesStageRna symbol
    Muscularstriatumskeletal  
    cell lineage
    cell lines
    fluid/secretion
    at STAGE
    PROTEIN
    PHYSICAL PROPERTIES globular
    STRUCTURE
    motifs/domains
    conjugated FlavoP
    mono polymer heteromer , dimer
    HOMOLOGY
    interspecies homolog to C.elegans f42a8.2
    Homologene
    FAMILY
  • succinate dehydrogenase/fumarate reductase iron-sulfur protein family
  • CATEGORY enzyme
    SUBCELLULAR LOCALIZATION     plasma membrane
        intracellular
    intracellular,cytoplasm,organelle,mitochondria,inner
    intracellular,cytoplasm,organelle,membrane
    basic FUNCTION
  • succinate-ubiquinone oxidoreductase, catalyzing the seventh step of the citric acid cycle (tricarboxylic acid cycle)
  • a crucial dosage-dependent role for SDHA, SDHB in oxygen homeostasis
  • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism organic acid
    signaling
    a component
  • complex II of mitochondrial respiratory chain, oxidative phosphorylation, (OXPHOS)
  • INTERACTION
    DNA
    RNA
    small molecule
    protein
  • SDHA and SDHB interacted specifically with SIRT3 (SIRT3 may be an important physiological regulator of SDH activity)
  • cell & other
    REGULATION
    ASSOCIATED DISORDERS
    corresponding disease(s) PGL4 , CWS2 , SDHBD
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    tumoral germinal mutation      
    in pheochromocytoma and paraganglioma
    tumoral       loss of function
    in pheochromocytoma and paraganglioma
    tumoral   LOH    
    in pheochromocytoma with high-risk of recurrence or malignancy
    tumoral germinal mutation      
    in Cowden and Cowden-like syndromes
    tumoral germinal mutation      
    in familial renal cell carcinoma, with early onset
    tumoral     --low  
    is limited to a distinctive subset of gastric wild-type gastrointestinal stromal tumours
    Susceptibility
  • pheochromocytoma, paraganglioma and early-onset renal cell carcinoma
  • Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS