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Titlebook: Organic Acids in Man; Analytical Chemistry R. A. Chalmers,A. M. Lawson Book 1982 R. A. Chalmers and A. M. Lawson 1982 Alanin.amino acid.bio

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Organic acids in physiological fluids other than urine and bloodect foetuses affected with some of the organic acidurias . by determination of enzyme activities of cultured amniotic cells or by direct chemical analysis of the amniotic fluid supernatant (Section 9.3). Few data, particularly quantitative, are available on the organic acid composition of amniotic f
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Disorders of branched-chain amino acid metabolism)-dependent transamination with 2-oxoglutarate in the first step and thiamin pyrophosphate-dependent oxidative decarboxylation in the second. The possibility of specificity in the first step is indicated by the occurrence of isolated hypervalinaemia (McKusick 27 710) (Dancis .., 1967) and two transa
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Disorders of propionate and methylmalonate metabolism4). The propionyl-CoA is further metabolized to methylmalonyl-CoA by a biotin-dependent carboxylase and subsequently via succinyl-CoA into the tricarboxylic acid cycle. .-Valine is also metabolized ultimately to methylmalonyl-CoA (Section 10.4), and thus these two branched-chain amino acids form the
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Disorders of ,-lysine metabolism3). An alternative pathway, via 2-keto-6-aminocaproic acid and pipecolic acid, also occurs and is the major degradative pathway for the unnatural .-lysine (Grove and Henderson, 1968). Hutzler and Dancis (1968) investigated the initial degradative step of lysine catabolism in humans and demonstrated
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