A Comparison of the Phenylalanine Content of the Hemoglobin of Normal and Phenylketonuric Individuals: Determination by Ion Exchange Chromatography1

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Abstract
The phenylalanine, tyrosine, glutamic acid and aspartic acid content of 2 samples of hemoglobin from normal individuals and 2 samples from phenylketonuric patients were compared. The determinations were made chromatographically on a 200-cm column of Dowex 2-X10, with 0.5 [image] acetic acid as a developer. No significant difference was noted between normal and phenylketonuric hemoglobin. There was less than 1 chance in 200 that the phenylalanine content of phenylketonuric hemoglobin exceeded that of normal hemoglobin by as much as 1 residue of phenylalanine, and less than 1 chance in 20 that phenylketonuric hemoglobin had 1 residue less of tyrosine, glutamic acid, or aspartic acid. Hence the experimental evidence suggests that the phenylalanine toxicity in phenylketonuria is not associated with any abnormal accumulation of phenylalanine in hemoglobin.