Target Information
Target General Infomation | |||||
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Target ID |
T76213
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Former ID |
TTDI01963
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Target Name |
Phenylalanine hydroxylase
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Gene Name |
PAH
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Synonyms |
Phe4monooxygenase; Phenylalanine4hydroxylase; PAH
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Target Type |
Successful
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Disease | Autoimmune diabetes [ICD10: E08-E13] | ||||
Genetic disease [ICD10: Q00-Q99] | |||||
Hyperphenylalaninemia [ICD10: E70.1] | |||||
Liver failure [ICD10: K72.9] | |||||
Phenylketonuria [ICD10: E70.0] | |||||
BioChemical Class |
Oxidoreductases acting on paired donors
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UniProt ID | |||||
EC Number |
EC 1.14.16.1
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Sequence |
MSTAVLENPGLGRKLSDFGQETSYIEDNCNQNGAISLIFSLKEEVGALAKVLRLFEENDV
NLTHIESRPSRLKKDEYEFFTHLDKRSLPALTNIIKILRHDIGATVHELSRDKKKDTVPW FPRTIQELDRFANQILSYGAELDADHPGFKDPVYRARRKQFADIAYNYRHGQPIPRVEYM EEEKKTWGTVFKTLKSLYKTHACYEYNHIFPLLEKYCGFHEDNIPQLEDVSQFLQTCTGF RLRPVAGLLSSRDFLGGLAFRVFHCTQYIRHGSKPMYTPEPDICHELLGHVPLFSDRSFA QFSQEIGLASLGAPDEYIEKLATIYWFTVEFGLCKQGDSIKAYGAGLLSSFGELQYCLSE KPKLLPLELEKTAIQNYTVTEFQPLYYVAESFNDAKEKVRNFAATIPRPFSVRYDPYTQR IEVLDNTQQLKILADSINSEIGILCSALQKIK |
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Drugs and Mode of Action | |||||
Pathways | |||||
BioCyc Pathway | Phenylalanine degradation/tyrosine biosynthesis | ||||
KEGG Pathway | Phenylalanine metabolism | ||||
Phenylalanine, tyrosine and tryptophan biosynthesis | |||||
Metabolic pathways | |||||
Biosynthesis of amino acids | |||||
PathWhiz Pathway | Phenylalanine and Tyrosine Metabolism | ||||
WikiPathways | SIDS Susceptibility Pathways | ||||
Biogenic Amine Synthesis | |||||
Metabolism of amino acids and derivatives | |||||
Abnormal metabolism in phenylketonuria | |||||
References | |||||
Ref 522703 | ClinicalTrials.gov (NCT00925054) Dose-Finding Study to Evaluate the Safety, Efficacy, & Tolerability of Multiple Doses of rAvPAL-PEG in Subjects With PKU. U.S. National Institutes of Health. | ||||
Ref 532742 | Single-dose, subcutaneous recombinant phenylalanine ammonia lyase conjugated with polyethylene glycol in adult patients with phenylketonuria: an open-label, multicentre, phase 1 dose-escalation trial. Lancet. 2014 Jul 5;384(9937):37-44. |
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