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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).
SLC7A7 SLC3A2 GOT1 IL4I1 TAT PAH GOT1 TAT IL4I1 YARS FARSA FARSB HPD HGD GSTZ1 FAH L-Tyrosine Fumaric acid Acetoacetic acid L-Phenylalanine L-Phenylalanine L-Tyrosine Oxoglutaric acid Phenylpyruvic acid L-Glutamic acid Water Oxygen Ammonia Hydrogen peroxide Oxoglutaric acid L-Glutamic acid L-erythro- tetrahydrobiopterin Oxygen 4a- Hydroxytetrahydrobiopterin Oxoglutaric acid 4- Hydroxyphenylpyruvic acid L-Glutamic acid Oxoglutaric acid L-Glutamic acid Water Oxygen Ammonia Hydrogen peroxide Adenosine triphosphate Adenosine monophosphate Pyrophosphate Adenosine triphosphate Adenosine monophosphate Pyrophosphate Oxygen Homogentisic acid Carbon dioxide Oxygen Maleylacetoacetic acid 4- Fumarylacetoacetic acid Water tRNA(Tyr) L-Tyrosyl- tRNA(Tyr) tRNA(Phe) L- Phenylalanyl- tRNA(Phe) Catecholamine Biosynthesis Tyrosine Metabolism Citric Acid Cycle