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PHENYLALANINE HYDROXYLASE DEFICIENCY IN THE PERIPHERAL LYMPHOCYTES FROM PHENYLKETONURIA
Author(s) -
Yoshii Tamiko,
Toda Kiyoshi,
Kobori Tatsuji
Publication year - 1977
Publication title -
the journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.9
H-Index - 65
eISSN - 1346-8138
pISSN - 0385-2407
DOI - 10.1111/j.1346-8138.1977.tb01010.x
Subject(s) - phenylalanine , phenylalanine hydroxylase , tyrosine , peripheral , tyrosine hydroxylase , enzyme , chemistry , endocrinology , medicine , biochemistry , amino acid
A radiograph of the incorporation of 3 H‐phenylalanine and 3 H‐tyrosine into cultured lymphocytes in a phenylketonuria patient indicates phenylalanine hydroxylase deficiency in the peripheral lymphocytes. Cultured lymphocytes from the patient showed much less uptake of 3 H‐phenylalanine and a slightly higher incorporation of 3 H‐tyrosine than the control. It is possible that a lack of phenylalanine hydroxylase in the peripheral lymphocytes of PKU patients prevents oxidation of phenylalanine to tyrosine.

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