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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
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