Excretion of Amino Acids in Cystinuria

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Abstract
The amino acid content of the urine of five cystinurics has been determined employing chromatography on columns of the ion exchange resin, Dowex-50. In every case the amount of arginine, lysine, ornithine, and cystine was found to be from 50 to 100 fold the normal level. The quantity of isoleucme was about twice the normal, whereas the taurine output was diminished to about 1/3 or less of normal. All other ninhydrin-positive constituents were in the normal range. The relative molar excretion of ornithine, cystine, arginine, and lysine, which was found to be about 1:1:2:4, is quite similar from one subject to the next despite the haphazard nature of the case material and the absence of any dietary control. The results suggest the hypothesis that cystinuria involves an enzymatic defect which affects simultaneously at some site or sites (probably the kidney) some phase of the metabolism of arginine, lysine, ornithine, and the sulfur amino acids and perhaps also isoleucine and taurine.

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