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No. Drug (Description in trials) DrugBank KEGG DRUG KEGG GENES KEGG PATHWAY Disease ID
1(S)-2-amino-6-(2-hydroxypropanoyl)-7,8-dihydropteridin-4(3H)-one----[1] 240
25,6,7,8-tetrahydrobiopterin[1] Sapropterin[1] D08505 --[1] 240
36R-BH4[1] Sapropterin[1] D08505 --[3] 86, 124, 240
4A16AX07----[1] 240
5Amino acids[1] Amino acids---[3] 70, 240, 298
6Behavioral: Neuro-psychological Assessment----[1] 240
7Behavioral: phenylalanine restricted diet[1] Phenylalanine[1] D00021 --[1] 240
8Behavioral: Restricted phenylalanine diet[1] Phenylalanine[1] D00021 --[1] 240
9BH4----[5] 51, 86, 124, 240, 299
10Blood samples----[9] 13, 41, 46, 51, 61, 97, 227, 240, 271
11BMN 165----[1] 240
12BMN 165 (rAvPAL-PEG)[1] Pegvaliase[1] D11077 [1] PAH 💬[5] Biosynthesis of amino acids, Folate biosynthesis, Metabolic pathways, Phenylalanine metabolism, Phenylalanine, tyrosine and tryptophan biosynthesis 💬[1] 240
13BMN 307----[1] 240
14BMN165 20mg/day----[1] 240
15BMN165 40mg/day----[1] 240
16CDX----[2] 222, 240
17CDX 6114----[1] 240
18CNSA-001----[1] 240
19Cohort 1 0.225g----[1] 240
20Cohort 2 0.75g----[1] 240
21Cohort 3 2.25 g----[1] 240
22Corn[1] Corn---[2] 46, 240
23Corn/soy oil[1] Corn---[1] 240
24Diagnostic Test: Whole body Phe oxidation testing----[1] 240
25Diet----[3] 97, 240, 299
26Dietary Supplement for PKU patients----[1] 240
27Docosahexaenoic acid[1] Doconexent---[7] 6, 49, 90, 94, 240, 299, 301
28Free amino acids intake[1] Amino acids---[1] 240
29Glycomacropeptide-based protein substitute----[1] 240
30Hepatocyte Transplant----[1] 240
31High oleic sunflower oil[1] Sunflower oil[1] D09006 --[1] 240
32HMI-103----[1] 240
33Immunosuppression----[1] 240
34KUVAN[1] Sapropterin[1] D08505 --[1] 240
35Kuvan 100mg soluble tablet[1] Sapropterin[1] D08505 --[1] 240
36KuvanTM Therapy----[1] 240
37Kuvan®[1] Sapropterin[1] D08505 --[1] 240
38Kuvan® (sapropterin dihydrochloride)[1] Sapropterin[1] D08505 --[1] 240
39Large Neutral Amino Acid Therapy----[1] 240
40Liver biopsy----[4] 93, 171, 231, 240
41Low calorie protein substitute----[1] 240
42Medikinet----[1] 240
43Microalgal oil----[1] 240
44Moxifloxacin[1] Moxifloxacin[2] D00874 ,
D08237
--[9] 2, 6, 46, 49, 85, 86, 113, 240, 299
45N.a.----[10] 3, 6, 8, 13, 46, 96, 97, 240, 256, 271
46Nap----[2] 96, 240
47Other: Diet----[1] 240
48Other: Diet treatment----[1] 240
49Other: Liver Evaluation----[1] 240
50Other: Meal Challenge----[1] 240
51Other: Normal (control) diet----[1] 240
52Other: Phenylalanine (Phe)-restricted diet[1] Phenylalanine[1] D00021 --[1] 240
53Other: Routine----[1] 240
54Palynziq[1] Pegvaliase[1] D11077 [1] PAH 💬[5] Biosynthesis of amino acids, Folate biosynthesis, Metabolic pathways, Phenylalanine metabolism, Phenylalanine, tyrosine and tryptophan biosynthesis 💬[1] 240
55PEG----[12] 6, 13, 41, 46, 65, 78, 96, 97, 140, 144, 240, 299
56PEGVALIASE[1] Pegvaliase[1] D11077 [1] PAH 💬[5] Biosynthesis of amino acids, Folate biosynthesis, Metabolic pathways, Phenylalanine metabolism, Phenylalanine, tyrosine and tryptophan biosynthesis 💬[1] 240
57Phe----[2] 75, 240
58Phenoptin----[2] 124, 240
59Phenylalanine[1] Phenylalanine[1] D00021 --[1] 240
60Phenylalanine restricted diet[1] Phenylalanine[1] D00021 --[1] 240
61Phenylalanine-free protein substitute in tablet form (XPhe minis)[1] Phenylalanine[1] D00021 --[1] 240
62Phenylalanine-free protein substitute tablets[1] Phenylalanine[1] D00021 --[1] 240
63Phenylketonuria-type diet----[1] 240
64PKU Explore----[1] 240
65PKU express plus----[1] 240
66PKU Sphere----[1] 240
67PKU sphere liquid----[1] 240
68PKU Start----[1] 240
69PKU/TYR GMP Protein Substitute----[1] 240
70Protein intake----[1] 240
71PTC923----[1] 240
72Radiation: Preparative Radiation Therapy----[1] 240
73RAvPAL-PEG[1] Pegvaliase[1] D11077 [1] PAH 💬[5] Biosynthesis of amino acids, Folate biosynthesis, Metabolic pathways, Phenylalanine metabolism, Phenylalanine, tyrosine and tryptophan biosynthesis 💬[1] 240
74RAvPAL-PEG 0.001 mg/kg[1] Pegvaliase[1] D11077 [1] PAH 💬[5] Biosynthesis of amino acids, Folate biosynthesis, Metabolic pathways, Phenylalanine metabolism, Phenylalanine, tyrosine and tryptophan biosynthesis 💬[1] 240
75RAvPAL-PEG 0.003 mg/kg[1] Pegvaliase[1] D11077 [1] PAH 💬[5] Biosynthesis of amino acids, Folate biosynthesis, Metabolic pathways, Phenylalanine metabolism, Phenylalanine, tyrosine and tryptophan biosynthesis 💬[1] 240
76RAvPAL-PEG 0.01 mg/kg[1] Pegvaliase[1] D11077 [1] PAH 💬[5] Biosynthesis of amino acids, Folate biosynthesis, Metabolic pathways, Phenylalanine metabolism, Phenylalanine, tyrosine and tryptophan biosynthesis 💬[1] 240
77RAvPAL-PEG 0.03 mg/kg[1] Pegvaliase[1] D11077 [1] PAH 💬[5] Biosynthesis of amino acids, Folate biosynthesis, Metabolic pathways, Phenylalanine metabolism, Phenylalanine, tyrosine and tryptophan biosynthesis 💬[1] 240
78RAvPAL-PEG 0.1 mg/kg[1] Pegvaliase[1] D11077 [1] PAH 💬[5] Biosynthesis of amino acids, Folate biosynthesis, Metabolic pathways, Phenylalanine metabolism, Phenylalanine, tyrosine and tryptophan biosynthesis 💬[1] 240
79RTX----[5] 11, 46, 65, 222, 240
80RTX-134----[1] 240
81Sapropterin[1] Sapropterin[1] D08505 --[3] 86, 124, 240
82Sapropterin (Kuvan)[1] Sapropterin[1] D08505 --[1] 240
83SAPROPTERIN DIHYDROCHLORIDE[1] Sapropterin[1] D08505 --[1] 240
84Sapropterin dihydrochloride, 6R-BH4, tetrahydrobiopterin[1] Sapropterin[1] D08505 --[1] 240
85Sapropterin Dihydrocholoride[1] Sapropterin[1] D08505 --[1] 240
86SAPROPTERIN HYDROCHLORIDE[1] Sapropterin[1] D08505 --[1] 240
87Sapropterin, sapropterin dihydrochloride[1] Sapropterin[1] D08505 --[1] 240
88Sapropterin, sapropterin hydrochloride[1] Sapropterin[1] D08505 --[1] 240
89Sapropterindihydrochloride----[1] 240
90Saproterin dihydrochloride----[1] 240
91Sunflower oil[1] Sunflower oil[1] D09006 --[3] 46, 240, 299
92SYNB1618----[1] 240
93SYNB1934----[1] 240
94Synergy----[1] 240
95T1401----[1] 240
96Tetrahydrobiopterin[1] Sapropterin[1] D08505 --[1] 240
97Tetrahydrobiopterin (BH4)[1] Sapropterin[1] D08505 --[1] 240