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FLASH GENE
Symbol BCAT2 contributors: mct/npt/pgu - updated : 02-05-2013
HGNC name branched chain aminotransferase 2, mitochondrial
HGNC id 977
Location 19q13.33      Physical location : 49.298.319 - 49.314.320
Synonym name
  • placental tissue protein 18
  • branched-chain amino acid aminotransferase
    • Synonym symbol(s) BCT2, PP18a, BCAM, ECA40, PP18, BCAT, BCAT(m)
    EC.number 2.6.1.42, 2.6.1.26
    DNA
    TYPE functioning gene
    STRUCTURE 15.98 kb     11 Exon(s)
    10 Kb 5' upstream gene genomic sequence study
    MAPPING cloned Y linked N status provisional
    RNA
    TRANSCRIPTS type messenger
    identificationnb exonstypebpproduct
    ProteinkDaAAspecific expressionYearPubmed
    11 - 1616 - 392 ubiquitously 2001 1117082
  • PP18a
  • entirely identical to the mitochondrial branched-chain aminotransferase
    • - - 1340 33.77 300 in smaller amounts in nearly all tissues 2001 1117082
    PP18b
    EXPRESSION
    Type
       expressed in (based on citations)
    organ(s)
    SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
    Cardiovascularvessel     Homo sapiensAdult
    cells
    SystemCellPubmedSpeciesStageRna symbol
    Cardiovascularendothelial cell Homo sapiensAdult
    cell lineage
    cell lines
    fluid/secretion
    at STAGE
    physiological period pregnancy
    Text placenta
    PROTEIN
    PHYSICAL PROPERTIES
    STRUCTURE
    motifs/domains
  • small domain (1-175)
  • a large domain (176-365)
  • a 27 AAs mitochondrial targeting sequence
  • a CXXC motif likely involved in regulation by redox status, with thiols groups (C315 appears to be the sensor for redox regulation of BCAT activity, whereas C318 acts as the "resolving cysteine", allowing for reversible formation of a disulfide bond)
    • mono polymer homomer , dimer
    HOMOLOGY
    Homologene
    FAMILY
  • class-IV pyridoxal-phosphate-dependent aminotransferase family
    • CATEGORY enzyme
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm,organelle,mitochondria
    intracellular,cytoplasm,cytosolic
    basic FUNCTION
  • catalyzes the transamination of the essential branched chain amino acids (leucine, isoleucine and valine) with alpha-ketoglutarate
  • BCAT1, BCAT2 are novel biomarkers for cytosolic and mitochondrial oxidative stress
  • role in metabolizing excess glutamate may factor more prominently
    • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism
    signaling
    a component
    INTERACTION
    DNA
    RNA
    small molecule cofactor,
  • pyridoxal phosphate
    • protein
    cell & other
    REGULATION
    Other redox-linked regulation of its activity by a peroxide sensitive CXXC center
    ASSOCIATED DISORDERS
    ANIMAL & CELL MODELS