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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] Non-proteinogenic BCAAs include 2-aminoisobutyric acid and alloisoleucine. Leucine ...
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Branched-chain amino acids (BCAA) are ubiquitous in many organisms, comprising 35% of all proteins and 40% of the amino acids required in all mammals. [13] Mammalian BCATs come in two isoforms: cytosolic (BCATc) and mitochondrial (BCATm). The isoforms share 58% homology, [15] but vary in location and catalytic efficiency.
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This enzyme catalyzes the reversible transamination of branched-chain alpha-keto acids (BCKAs) to the branched-chain amino acids (BCAAs) valine, leucine and isoleucine, which are essential for cell growth. In humans, its primary role is the deamination of BCAAs, as humans lack the enzymes for de novo synthesis of BCKAs.
The buildup of these BCAAs will lead to the maple syrup odor in earwax and urine that is associated with MSUD. [14] The BCKAD complex begins by breaking down leucine, isoleucine, and valine through the use of branch-chain aminotransferase (BCAT) into their relevant α-ketoacids. The second step involves the conversion of α-ketoacids into ...
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