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Determination of Phe-hydroxylase in PKU and Hyperphe-aemia

The activity of phe-hydroxylase was determined in liver needle biopsy material from patients with elevated phe levels.The reaction mixture for the enzyme assay was: phosphate buffer 150mM, phe 0.1mM, phe 14C 0.2μCi, dithiothreitol 2mM, biopterin 0.025mM.The extract was preincubated with lysolecithin (1mM) for 10min, the reaction stopped by boiling. Phe and tyr were separated by TLC. 14-C-activity was determined by direct scanning and counted in a liquid scintillation counter.So far we have observed 4 types of enzyme activity: 1) Control liver samples of non-metabolic disorders transformed 100μmoles/g−1 protein/60min−1 from phe to tyr. 2) Socalled hyperphe-aemia (phe levels around 10 mg%) had 10-20% normal activity. 3) A third group had 1-6%. 2 pairs of siblings also showed this residual activity. One sib in each family had not been treated (late discovered) and was mentally retarded, the other developed normally under treatment. 4) “Classical” PKU had no detectible hydroxylase activity. -This study intends to differentiate PKU from other hyperphe-aemias which perhaps may remain untreated.