Reactome | leu, ile, or val + alpha-ketoglutarate <=> a-ketoisocaproate, a-keto-b-methylvalerate, or a-ketoisovalerate + glutamate [BCAT2]

leu, ile, or val + alpha-ketoglutarate <=> a-ketoisocaproate, a-keto-b-methylvalerate, or a-ketoisovalerate + glutamate [BCAT2]

Stable Identifier

R-HSA-70724

Type

Reaction

Species

Homo sapiens

Compartment

mitochondrial matrix

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leu, ile, or val + alpha-ketoglutarate <=> a-ketoisocaproate, a-keto-b-methylvalerate, or a-ketoisovalerate + glutamate [BCAT2]

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Mitochondrial branched-chain-amino-acid aminotransferase (BCAT2) catalyzes the reversible reactions of leucine, isoleucine, or valine with alpha-ketoglutarate (2-oxoglutarate) to form alpha-ketoisocaproate, alpha-keto-beta-methylvalerate, or a-ketoisovalerate, respectively, and glutamate (Bledsoe et al. 1997). The active enzyme is a homodimer (Yennawar et al. 2001, 2002). In the body, this enzyme is widely expressed but is especially abundant in muscle tissue.

Literature References

PubMed ID	Title	Journal	Year
9165094	Cloning of the rat and human mitochondrial branched chain aminotransferases (BCATm) Hutson, SM, Dawson, PA, Bledsoe, RK	Biochim Biophys Acta	1997
12269802	Crystal structures of human mitochondrial branched chain aminotransferase reaction intermediates: ketimine and pyridoxamine phosphate forms Farber, GK, Yennawar, HP, Conway, ME, Hutson, SM, Yennawar, NH	Biochemistry	2002
11264579	The structure of human mitochondrial branched-chain aminotransferase. Farber, GK, Conway, ME, Hutson, SM, Yennawar, NH, Dunbar, J	Acta Crystallogr D Biol Crystallogr	2001