Phenylalanine Derivatives Melanin

Phenylacetate L-Dopa Phenyllactate Phenylpyruvate

B PAH A

tyrosinase

T3, T4

Phenylalanine
C 1. 2. Tyrosine AT P-hydroxyphenylpyruvate hydroxylase

Tyrosine
E

Tyrosine hydroxylase

L-Dopa

Key A hyperphenylalaninemia B Classical/Variant PKU; A C Mutagenic PKU; A D Albinism E Tyrosinemia II

Dopamine

Homogentisate
F

Norepinephrine

F Alkaptonuria G Tyrosinemia I

Homogentisate oxidase

Epinephrine
G

Adrenal glands (and neurons)

Fumarylacetoacetate
Melanocytes Fumarylacetotacetate hydrolase Thyroid gland

Neurons

O2 H2O BH4 BH2 Fumarate + Acetoacetate

Liver - Toxic - See adjacent reaction * - Sometimes, DHFR catalyzes this reaction. Under those conditions, NADH is used instead. NADP+ DHPR*

NADPH + +H