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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
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
Physical map
GRWD1 19q13.33 glutamate-rich WD repeat containing 1 KCNJ14 19q13 potassium inwardly-rectifying channel, subfamily J, member 14 PSCD2 19q13.3 pleckstrin homology, Sec7 and coiled-coil domains 2 (cytohesin-2) KIAA1883 SULT2B1 19q13.3 sulfotransferase family, cytosolic, 2B, member 1 FLJ20200 19q13.33 hypothetical protein FLJ20200 SPACA4 19q13.3-14 sperm acrosome associated 4 RPL18 19q13.3 ribosomal protein L18 SPHK2 19q13.2 sphingosine kinase 2 DBP 19q13.3 D site of albumin promoter (albumin D-box) binding protein CA11 19q13.3 carbonic anhydrase XI LOC126147 19q13.33 hypothetical protein BC018697 FUT2 19q13.3 fucosyltransferase 2 (secretor status included) FLJ36070 19q13.33 hypothetical protein FLJ36070 RAIN 19q13.33 Ras-interacting protein MGC34799 19q13.33 hypothetical protein MGC34799 FUT1 19q13.3 fucosyltransferase 1 (galactoside 2-alpha-L-fucosyltransferase, Bombay phenotype included) FGF21 19q13.1-qter fibroblast growth factor 21 BCAT2 19q13.3 branched chain aminotransferase 2, mitochondrial DHRS10 19q13.33 dehydrogenase/reductase (SDR family) member 10 PLEKHA4 19q13.33 pleckstrin homology domain containing, family A (phosphoinositide binding specific) member 4 PPP1R15A 19q13.2 protein phosphatase 1, regulatory (inhibitor) subunit 15A TULP2 19q13.1 tubby like protein 2 NUCB1 19q13.32 nucleobindin 1 DHDH 19q13.3 dihydrodiol dehydrogenase (dimeric) BAX 19q13.3-q13.4 BCL2-associated X protein FTL 19q13.3 ferritin, light polypeptide GYS1 19q13.3 glycogen synthase 1 (muscle) RUVBL2 19q13.3 RuvB-like 2 (E. coli)
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