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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
    Physical map
    CPB2 13q14.11 carboxypeptidase B2 (plasma, carboxypeptidase U) LCP1 13q14.3 lymphocyte cytosolic protein 1 (L-plastin) LOC220416 13q14.11 similar to Leucine-rich repeat protein SHOC-2 (Ras-binding protein Sur-8) LOC390403 13 similar to hypothetical protein FLJ20457 C13orf18 13q14.11 chromosome 13 open reading frame 18 LOC81993 13q14-q21 COX17 pseudogene CHDC1 13q14.11 calponin homology (CH) domain containing 1 ESD 13q14.1-q14.2 esterase D/formylglutathione hydrolase HTR2A 13q14.3 5-hydroxytryptamine (serotonin) receptor 2A SUCLA2 13q12.2-q13.3 succinate-CoA ligase, ADP-forming, beta subunit NUDT15 13q14.12 nudix (nucleoside diphosphate linked moiety X)-type motif 15 VDRIP 13q14.12 vitamin D receptor interacting protein ITM2B 13q14.3 integral membrane protein 2B RB1 13q14.2 retinoblastoma 1 (including osteosarcoma) P2RY5 13q14 purinergic receptor P2Y, G-protein coupled, 5 CHC1L 13q14.3 chromosome condensation 1-like CYSLTR2 13q14.12-p21.1 cysteinyl leukotriene receptor 2 LOC338099 13q13 proteasome activator subunit 2 pseudogene KIAA0970 13q14.11 proteasome activator subunit 2 pseudogene RAD17P2 13q14.3 RAD17 homolog (S. pombe) pseudogene 2 COX7CP1 13q14-q21 cytochrome c oxidase subunit VIIc pseudogene 1 LOC387924 13 similar to hypothetical protein FLJ10826 GPR38 13q14-q21 G protein-coupled receptor 38 CDADC1 13q14.12 cytidine and dCMP deaminase domain containing 1 FLJ12577 13q14.12 hypothetical protein FLJ12577 SETDB2 13q14 SET domain, bifurcated 2 PHF11 13q14.12 PHD finger protein 11 RCBTB1 13q14 regulator of chromosome condensation (RCC1) and BTB (POZ) domain containing protein 1 ARLTS1 EBRP 13q12-q13 emopamil binding related protein, delta8-delta7 sterol isomerase related protein KPNA3 13q14.3 karyopherin alpha 3 (importin alpha 4)
    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