THE METABOLISM OF SOME BRANCHED CHAIN ALIPHATIC ACIDS
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چکیده
منابع مشابه
Branched Chain Amino Acids: Beyond Nutrition Metabolism.
Branched chain amino acids (BCAAs), including leucine (Leu), isoleucine (Ile), and valine (Val), play critical roles in the regulation of energy homeostasis, nutrition metabolism, gut health, immunity and disease in humans and animals. As the most abundant of essential amino acids (EAAs), BCAAs are not only the substrates for synthesis of nitrogenous compounds, they also serve as signaling mole...
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Branched-chain fatty acids are common constituents of the lipids of bacteria and animals, although they are rarely found in the integral lipids of higher plants. Normally, the fatty acyl chain is saturated and the branch is a methyl-group. However, unsaturated branched-chain fatty acids are found in marine animals, and branches other than methyl may be present in microbial lipids. The most comm...
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Genetic disorders of BCAA metabolism produce amino acidopathies and various forms of organic aciduria with severe clinical consequences. A metabolic block in the oxidative decarboxylation of BCAA caused by mutations in the mitochondrial branched-chain a-keto acid dehydrogenase complex (BCKDC) results in Maple Syrup Urine Disease (MSUD) or branched-chain ketoaciduria. There are presently five kn...
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The three branched-chain amino acids (BCAAs) are the most hydrophobic of the amino acids and play crucial roles in determining the structures of globular proteins as well as the interaction of the transmembrane domains of membranous proteins with phospholipid bilayers. However, the three BCAAs do not behave identically. In terms of protein secondary structure, valine and isoleucine exhibit a de...
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In the brain, catabolism of the branched-chain amino acids (BCAAs) provides nitrogen for the synthesis of glutamate and glutamine. Glutamate is formed through transfer of an amino group from BCAA to alpha-ketoglutarate in reaction catalyzed by branched-chain aminotransferases (BCAT). There are two isozymes of BCAT: cytosolic BCATc, which is found in the nervous system, ovary, and placenta, and ...
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ژورنال
عنوان ژورنال: Journal of Biological Chemistry
سال: 1949
ISSN: 0021-9258
DOI: 10.1016/s0021-9258(18)56945-3