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A branched-chain amino acid (BCAA) is an amino acid having an aliphatic side-chain with a branch (a central carbon atom bound to three or more carbon atoms). Among the proteinogenic amino acids , there are three BCAAs: leucine , isoleucine , and valine . [ 1 ]
In Corynebacterium glutamicum, BrnFE exports branched chain amino acids isoleucine, leucine, and valine, as well as methionine and homoserine. [3] [5] [8] The greatest induction of BrnFE expression occurs under high methionine concentrations, suggesting that methionine may actually be the native substrate rather than a branched-chain amino acid ...
The BCAT enzyme catalyzes the conversion of BCAAs and α-ketoglutarate into branched chain α-keto acids and glutamate. The structure to the right of branched chain amino acid aminotransferase was found using X-ray diffraction with a resolution of 2.20 Å. The branched-chain amino acid aminotransferase found in this image was isolated from ...
Branched-chain keto acid dehydrogenase kinase deficiency (BCKDK deficiency) is a disease resulting from mutations of the BCKDK gene. Patients with BCKDK deficiency have low levels of branched chain amino acids (BCAA) in their organism due to accelerated breakdown of these essential amino acids.
In animal tissue, BCKDC catalyzes an irreversible step [2] in the catabolism of the branched-chain amino acids L-isoleucine, L-valine, and L-leucine, acting on their deaminated derivatives (L-alpha-keto-beta-methylvalerate, alpha-ketoisovalerate, and alpha-ketoisocaproate, respectively) and converting them [3] to α-Methylbutyryl-CoA, Isobutyryl-CoA and Isovaleryl-CoA respectively.
The second major step in the catabolism of the branched-chain amino acids (isoleucine, leucine, and valine) is catalyzed by the branched-chain alpha-keto acid dehydrogenase complex (BCKD; EC 1.2.4.4), an inner-mitochondrial enzyme complex that consists of 3 catalytic components: a heterotetrameric (alpha2, beta2) branched-chain alpha-keto acid decarboxylase (E1), a homo-24-meric dihydrolipoyl ...
Amino acids are the chemical building blocks of protein. They're found in protein-rich foods, or there are specific supplements such as branched-chain amino acids and essential amino acids.
BCKDK regulates the activity of branched-chain α-ketoacid dehydrogenase complex (BCKD) through phosphorylation and inactivation. This inactivation results in increased branched-chain amino acids (BCAA), which is seen to reduce oxidative stress; however, having too much BCAA has been proven to be toxic to humans. Therefore, BCKDK is a vital ...
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