Pediat. Res. 4: 89-95 (1970)
ExtractPipecolic 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 fluids. Pipecolic acid was present in the urine from: four premature infants (6-23 days old) 1.2-8.1 ,«g/ m g creatinine; two of four term infants (3-5 days old) 1.3 and 2.1 ,«g/mg creatinine; and one of four infants (4-11 months old) 2.1 /jg/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 excreted in the urine were: 3.1 ^g/mg creatinine (4-year-old girl); 5.2 //g/rng creatinine (6-year-old boy); 6.2 /"g/mg creatinine (9-year-old girl); and 4.8 ,ug/mg creatinine (12-year-old girl).
SpeculationThe 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 pipecolic acid excreted by young infants and by children with hyperlysinemia indicate that a degradation pathway for lysine, via pipecolic acid to a-aminoadipic acid, is operative in man.
Introductionamino acids by infants, children, and adults [2,4,8-10, 14, 16, 19, 21-23, 25, 26, 28, 30, 36, 39, 41-45, 47, 48] Pipecolic acid has been shown to be a product of lysine make no mention of pipecolic acid as a normal urinary metabolism in rats [31][32][33], guinea pigs [5,20,34], constituent, but JAGENBURG [18] detected pipecolic and turkeys [6,7]. It has been detected in plants [53] acid in urine from infants less than 1 year old; however, and is derived from lysine in some of them [15,24]. he was unable to find it in urine of adults. Pipecolic Certain microorganisms form pipecolic acid from a-acid also has been found in the urine of a child with aminoadipic acid [3]; others derive it from lysine by hyperlysinemia [49] and in urine of patients with pathways similar to those described for mammals [20, hyperthyroidism [38]. GHADIMI et al. [13], however,35].attempting to detect pipecolic acid in urine from norReports of the presence of pipecolic acid in human mal premature infants, infants, and adults, and from urine are conflicting. Numerous studies of excretion of patients with hyperlysinemia were unsuccessful, and