Y+L amino acid
transporter 1
4F2
cell-surface
antigen heavy
chain
Aspartate
aminotransferase,
cytoplasmic
L-amino-acid
oxidase
Tyrosine
aminotransferase
Phenylalanine-
4-hydroxylase
Aspartate
aminotransferase,
cytoplasmic
Tyrosine
aminotransferase
L-amino-acid
oxidase
Tyrosine--tRNA
ligase,
cytoplasmic
Phenylalanine-
-tRNA ligase
4-
hydroxyphenylpyruvate
dioxygenase
Homogentisate
1,2-dioxygenase
Maleylacetoacetate
isomerase
Fumarylacetoacetase
L-Tyrosine
Fumaric acid
Acetoacetic acid
L-Phenylalanine
L-Phenylalanine
L-Tyrosine
Oxoglutaric acid
Phenylpyruvic acid
L-Glutamic acid
H2O
O2
NH3
H2O2
Oxoglutaric acid
L-Glutamic acid
L-erythro-tetrahydrobiopterin
O2
4a-Hydroxytetrahydrobiopterin
Oxoglutaric acid
4-Hydroxyphenylpyruvic acid
L-Glutamic acid
Oxoglutaric acid
L-Glutamic acid
H2O
O2
NH3
H2O2
ATP
AMP
PPi
ATP
AMP
PPi
O2
Homogentisic acid
CO2
O2
Maleylacetoacetic acid
4-Fumarylacetoacetic acid
H2O
Fe2+
Fe3+
Calcium
Magnesium
tRNA(Tyr)
L-Tyrosyl-
tRNA(Tyr)
tRNA(Phe)
L-
Phenylalanyl-
tRNA(Phe)
Catecholamine
Biosynthesis
Tyrosine
Metabolism
Citric Acid
Cycle
Pyridoxal
5'-phosphate
FAD
Pyridoxal
5'-phosphate
Pyridoxal
5'-phosphate
Pyridoxal
5'-phosphate
FAD
Iron
Cytosol
Extracellular Space
Liver Hepatic Cell
Lysosome
Seizures, mental retardation
and intermittent ataxia
(occasional loss of balance
and coordination).