Method for Determination of Serum Phenylalanine with Use of the Kapeller-Adler Reaction FREE

RICHARD J. HENRY, M.D.; CHARLES SOBEL, B.S.; NEIL CHIAMORI, B.S.

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Received for publication Aug. 5, 1957; accepted Aug. 27.

Bio-Science Laboratories.

AMA Am J Dis Child. 1957;94(6):604-608. doi:10.1001/archpedi.1957.04030070016003.

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ABSTRACT

ABSTRACT | REFERENCES

The discovery that serum phenylalanine is increased in the metabolic disease phenylpyruvic oligophrenia has made desirable the availability of a relatively simple and accurate method for its determination in serum. This has become even more important since the observations that urine phenylpyruvic acid, which has been the commonly used test for this disease, may not be detected until the serum levels of phenylalanine exceed about 15 mg. per 100 cc.¹ and that infants less than one month old with this disease may not show the presence of phenylpyruvic acid in the urine, whereas the serum phenylalanine is elevated within five days.²

Microbiological methods ³ have been used, but these do not meet the requirement of simplicity. Udenfriend and Cooper⁴ have proposed a chemical method in which the phenylalanine is enzymatically decarboxylated to phenylethylamine by use of an extract of Streptococcus faecalis and then the phenylethylamine is quantitated

REFERENCES

ABSTRACT | REFERENCES

Armstrong, M.D., and Low, N. L.: Phenylketonuria: VIII. Relation Between Age, Serum Phenylalanine Level, and Phenylpyruvic-Acid Excretion , Proc. Soc. Exper. Biol. & Med. 94:142, 1957;.

Armstrong, M. D., and Binkley, E. L., Jr.: Studies on Phenylketonuria: V. Observations on a Newborn Infant with Phenylketonuria , Proc. Soc. Exper. Biol. & Med. 93:418, 1956;.

Borek, E.; Brecher, A.; Jervis, G. A., and Waelsch, H.: Oligophrenia Phenylpyruvica: II. Constancy of the Metabolic Error , Proc. Soc. Exper. Biol. & Med. 75:86, 1950;.

Udenfriend, S., and Cooper, J. R.: Assay of L-Phenylalanine as Phenylethylamine After Enzymatic Decarboxylation: Application to Isotopic Studies , J. Biol. Chem. 203:953, 1953;.

Kapeller-Adler, R.: Über eine neue Reaktion zur qualitativen und quantitativen Bestimmung des Phenylalanins , Biochem. Ztschr. 252:185, 1932;.

Block, R. J., and Bolling, D.: The Amino Acid Composition of Proteins and Foods: Analytical Methods and Results , Ed. 2, Springfield, Ill., Charles C Thomas, Publisher, 1951;, p. 137.

Armstrong, M. D., and Tyler, F. H.: Studies on Phenylketonuria: I. Restricted Phenylalanine Intake in Phenylketonuria , J. Clin. Invest. 34: 565, 1955;.

Stein, W. H., and Moore, S.: The Free Amino Acids of Human Blood Plasma , J. Biol. Chem. 211:915, 1954;.

Dunn, M. S.; Schott, H. F.; Frankl, W., and Rockland, L. B.: Investigations of Amino Acids, Peptides, and Proteins: XX. The Determination of Apparent Free Tryptophane in Blood by a Microbiological Method , J. Biol. Chem. 157:387, 1945;.

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