Gene Symbol: Bcat2
Description: branched chain aminotransferase 2, mitochondrial
Alias: Bcat(m), Bcat-2, Eca40, branched-chain-amino-acid aminotransferase, mitochondrial
Species: mouse
Products:     Bcat2

Top Publications

  1. She P, Reid T, Bronson S, Vary T, Hajnal A, Lynch C, et al. Disruption of BCATm in mice leads to increased energy expenditure associated with the activation of a futile protein turnover cycle. Cell Metab. 2007;6:181-94 pubmed
    ..These observations suggest that elevated BCAAs and/or loss of BCAA catabolism in peripheral tissues play an important role in regulating insulin sensitivity and energy expenditure. ..
  2. Hatazawa Y, Tadaishi M, Nagaike Y, Morita A, Ogawa Y, Ezaki O, et al. PGC-1?-mediated branched-chain amino acid metabolism in the skeletal muscle. PLoS ONE. 2014;9:e91006 pubmed publisher
    ..In C2C12 cells, the overexpression of PGC-1? significantly increased the expression of BCAT2 and BCKDH but not BCKDK...
  3. Ben Yosef T, Eden A, Benvenisty N. Characterization of murine BCAT genes: Bcat1, a c-Myc target, and its homolog, Bcat2. Mamm Genome. 1998;9:595-7 pubmed
  4. Wu J, Kao H, Li S, Stevens R, Hillman S, Millington D, et al. ENU mutagenesis identifies mice with mitochondrial branched-chain aminotransferase deficiency resembling human maple syrup urine disease. J Clin Invest. 2004;113:434-40 pubmed
    ..Metabolomics-guided screening, coupled with ENU mutagenesis, is a powerful approach in uncovering novel enzyme deficiencies and recognizing important pathways of genetic metabolic disorders. ..
  5. She P, Zhou Y, Zhang Z, Griffin K, Gowda K, Lynch C. Disruption of BCAA metabolism in mice impairs exercise metabolism and endurance. J Appl Physiol (1985). 2010;108:941-9 pubmed publisher
    ..Thus BCAA metabolism may regulate exercise capacity in mice. ..
  6. Purpera M, Shen L, Taghavi M, Munzberg H, Martin R, Hutson S, et al. Impaired branched chain amino acid metabolism alters feeding behavior and increases orexigenic neuropeptide expression in the hypothalamus. J Endocrinol. 2012;212:85-94 pubmed publisher
    ..These data thus suggest that either BCAAs do not act as physiological signals of protein status or the loss of BCAA metabolism within brain glia impairs the detection of protein balance. ..
  7. Lang C, Lynch C, Vary T. BCATm deficiency ameliorates endotoxin-induced decrease in muscle protein synthesis and improves survival in septic mice. Am J Physiol Regul Integr Comp Physiol. 2010;299:R935-44 pubmed publisher
  8. Zampieri T, Pedroso J, Furigo I, Tirapegui J, Donato J. Oral leucine supplementation is sensed by the brain but neither reduces food intake nor induces an anorectic pattern of gene expression in the hypothalamus. PLoS ONE. 2013;8:e84094 pubmed publisher
    ..hypothalamic gene expression and observed that leucine supplementation increased the expression of enzymes (BCAT1, BCAT2 and BCKDK) that metabolize branched-chain amino acids...
  9. Koike H, Zhang R, Ueno Y, Sekine K, Zheng Y, Takebe T, et al. Nutritional modulation of mouse and human liver bud growth through a branched-chain amino acid metabolism. Development. 2017;144:1018-1024 pubmed publisher

More Information


  1. Bledsoe R, Dawson P, Hutson S. Cloning of the rat and human mitochondrial branched chain aminotransferases (BCATm). Biochim Biophys Acta. 1997;1339:9-13 pubmed
    ..Finally, the nomenclature BCAT1 for the cytosolic gene and BCAT2 for the mitochondrial BCAT gene is proposed.
  2. Sewell W, Sparrow D, Smith A, Gonzalez D, Rappaport E, Dunwoodie S, et al. Cyclical expression of the Notch/Wnt regulator Nrarp requires modulation by Dll3 in somitogenesis. Dev Biol. 2009;329:400-9 pubmed publisher
    ..Towards identifying the role of Dll3 in regulating somitogenesis, Nrarp emerges as a potentially important regulator that requires Dll3 but not Lfng for normal function...
  3. Lynch C, Kimball S, Xu Y, Salzberg A, Kawasawa Y. Global deletion of BCATm increases expression of skeletal muscle genes associated with protein turnover. Physiol Genomics. 2015;47:569-80 pubmed publisher
    ..Mice lacking the mitochondrial branched-chain aminotransferase (BCATm/Bcat2), which interconverts leucine and α-ketoisocaproate, exhibit elevated protein turnover...
  4. Herman M, She P, Peroni O, Lynch C, Kahn B. Adipose tissue branched chain amino acid (BCAA) metabolism modulates circulating BCAA levels. J Biol Chem. 2010;285:11348-56 pubmed publisher
    ..These results demonstrate for the first time the capacity of adipose tissue to catabolize circulating BCAAs in vivo and that coordinate regulation of adipose-tissue BCAA enzymes may modulate circulating BCAA levels. ..
  5. Zimmerman H, Olson K, Chen G, Lynch C. Adipose transplant for inborn errors of branched chain amino acid metabolism in mice. Mol Genet Metab. 2013;109:345-53 pubmed publisher
    ..Therefore, subcutaneous fat transplantation may have merit as an adjunct to dietary treatment of MSUD. Additional studies are needed to further refine this approach. ..
  6. Lang C, Lynch C, Vary T. Alcohol-induced IGF-I resistance is ameliorated in mice deficient for mitochondrial branched-chain aminotransferase. J Nutr. 2010;140:932-8 pubmed publisher
    ..These data suggest that whereas the sustained elevation in plasma BCAA is not sufficient to ameliorate the catabolic effect of acute alcohol intoxication on muscle protein synthesis, it does improve the anabolic effect of IGF-I. ..