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. 2013 Aug;59(8):837-840.

Figure 2.

Figure 2

Metabolic pathway in individuals with PKU: Absence or severe reduction of PAH with normal BH4 resulting in accumulation of Phe and reduction of Tyr.

BH4 —tetrahydrobiopterin, PAH—phenylalanine hydroxylase, Phe—phenylalanine, PKU—phenylketonuria, Tyr—tyrosine.