Selected-GenAtlas references SOURCE GeneCards NCBI Gene Swiss-Prot Orphanet Ensembl
HGNC UniGene Nucleotide OMIM UCSC
Home Page
FLASH GENE
Symbol SDHA contributors: mct - updated : 07-01-2014
HGNC name succinate dehydrogenase complex, subunit A, flavoprotein (Fp)
HGNC id 10680
Corresponding disease
CMD1GG cardiomyopathy, dilated, 1GG
PGL5 hereditary paraganglioma 5
SDHAD succinate CoQ reductase deficiency
Location 5p15.33      Physical location : 218.355 - 256.812
Synonym name flavoprotein subunit of complex II
Synonym symbol(s) FPJ, SDH2, SDHF, FP, SDH1, CMD1GG
EC.number 1.3.5.1
DNA
TYPE functioning gene
STRUCTURE 38.46 kb     15 Exon(s)
10 Kb 5' upstream gene genomic sequence study
MAPPING cloned Y linked   status provisional
RNA
TRANSCRIPTS type messenger
identificationnb exonstypebpproduct
ProteinkDaAAspecific expressionYearPubmed
15 - 2405 - 664 cervical carcinoma and paraganglioma 2007 17376234
- - - - - in the Burkitt lymphoma and paraganglioma 2005 16195397
EXPRESSION
Type
   expressed in (based on citations)
organ(s)
SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
Digestiveliver    
cell lineage
cell lines
fluid/secretion
at STAGE
PROTEIN
PHYSICAL PROPERTIES globular
STRUCTURE
motifs/domains
  • characteristic cysteine triplet FAD binding domain
  • HOMOLOGY
    interspecies homolog to C.elegans c03g5.1
    Homologene
    FAMILY
    CATEGORY enzyme
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm,organelle,mitochondria,inner
    intracellular,cytoplasm,organelle,membrane
    intracellular,nucleus
    basic FUNCTION
  • catalyzing the seventh step of the citric acid cycle, i.e. oxidation of succinate to fumarase
  • play a role in mitochondrial oxidative phosphorylation
  • a crucial dosage-dependent role for SDHA, SDHB in oxygen homeostasis
  • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism
    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) SDHAD , PGL5 , CMD1GG
    Susceptibility to oligospermia
    Variant & Polymorphism SNP SDHA c.456+32G>A) showed significant genotype association with impairment of sperm production
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS