Abstract
Extract: Pipecolic acid has been found in urine from normal infants and from children with hyperlysinemia. The reaction of pipecolic acid with ninhydrin reagent, using separation procedures in automated ion-exchange systems for amino acid analysis, produced a color constant of low value (1.21 compared with 27.6 for leucine) that could be overlooked in routine analyses of physiological flusids. Pipecolic acid was present in the urine from: four premature infants (6–23 days old) 1.2–8.1 μg/mg creatinine; two of four term infants (3–5 days old) 1.3 and 2.1 μg/mg creatinine; and one of four infants (4–11 months old) 2.1 μ/mg. No pipecolic acid was found in the urines of four infants 14–30 months old. In patients with hyperlysinemia, the amounts of pipecolic acid excreated in the urine were: 3.1 μ/mg creatinine (4-year-old girl); 5.2 μ/mg creatinine (6-year-old boy); 6.2 μ/mg creatinine (9-year-old girl); and 4.8 μ/mg creatinine (12-year-old girl).
Speculation: The low color yield of pipecolic acid, when separated by standard automated ion-exchange analysis methods, may account for past failures to detect small amounts of this substance in physiological fluids. Amounts of popecolic acid excreated by yound infants and by children with hyperlysinemia indicate that a degradation pathway for lysine, via pipevolic acid to α-aminoadipic acid, is operative in man.
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Woody, N., Pupene, M. Excretion of Pipecolic Acid by Infants and by Patients with Hyperlysinemia. Pediatr Res 4, 89–95 (1970). https://doi.org/10.1203/00006450-197001000-00011
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DOI: https://doi.org/10.1203/00006450-197001000-00011
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