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FLASH GENE
Symbol SUCLA2 contributors: mct - updated : 03-11-2015
HGNC name succinate-CoA ligase, ADP-forming, beta subunit
HGNC id 11448
Corresponding disease
MDDS4 mitochondrial DNA depletion syndrome, encephalomyopathic form
Location 13q14.2      Physical location : 48.516.791 - 48.575.462
Synonym name
  • SCS-betaA for ATP specific succinyl CoA synthetase beta subunit precursor
  • subunit of the ADP-forming succinyl-CoA synthetase (SCS-A)
  • Synonym symbol(s) A-BETA, MTDPS5, SCS-betaA
    EC.number 6.2.1.5
    DNA
    TYPE functioning gene
    STRUCTURE 58.67 kb     11 Exon(s)
    10 Kb 5' upstream gene genomic sequence study
    MAPPING cloned Y linked N status provisional
    Map cen - D13S287 - D13S1764 - SUCLA2 - D13S1195 - D13S164 - qter
    RNA
    TRANSCRIPTS type messenger
    identificationnb exonstypebpproduct
    ProteinkDaAAspecific expressionYearPubmed
    11 - 2182 - 463 - 1998 9765291
    EXPRESSION
    Type ubiquitous
       expressed in (based on citations)
    organ(s)
    SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
    Cardiovascularheart    
    Digestiveesophagus   highly
     intestinesmall intestine  highly
    Endocrineadrenal gland   highly
    Nervousbrainforebraincerebral cortex highly Homo sapiens
    Reproductivemale systemprostate   
    Respiratorylung    
    tissue
    SystemTissueTissue level 1Tissue level 2LevelPubmedSpeciesStageRna symbol
    Muscularstriatumskeletal  
    cells
    SystemCellPubmedSpeciesStageRna symbol
    Nervousneuron Homo sapiens
    cell lineage
    cell lines
    fluid/secretion
    at STAGE
    PROTEIN
    PHYSICAL PROPERTIES
    STRUCTURE
    motifs/domains
    mono polymer homomer , dimer
    HOMOLOGY
    Homologene
    FAMILY succinate/malate coA ligase beta subunit family
    CATEGORY enzyme
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm,organelle,mitochondria,matrix
    basic FUNCTION
  • catalyzing the formation of succinate and ATP from succinyl-CoA and ADP in the tricarboxylic acid (TCA) cycle in a reversible manner
  • subunit of succinyl-coenzyme A synthase, the enzyme that reversibly synthesises succinyl-coenzyme A and ATP from succinate, coenzyme A and ADP in the Krebs cycle
  • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism
    signaling
    a component
    INTERACTION
    DNA
    RNA
    small molecule
    protein
  • bind to nucleoside diphosphate kinase, both in prokaryotes and in eukaryotes
  • unique interaction of SUCLA2 with ALAS2 is involved in an important erythroid-specific function
  • cell & other
    REGULATION
    ASSOCIATED DISORDERS
    corresponding disease(s) MDDS4
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    constitutional germinal mutation      
    associated to mitochondrial DNA (mtDNA) depletion syndrome (MTENC5)
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS