Page last updated: 2024-10-06
Phenylketonuria
Proteins (11)
| Protein | Synonyms | Taxonomy |
|---|
| Tyrosine--tRNA ligase, cytoplasmic | EC 6.1.1.1; Tyrosyl-tRNA synthetase; TyrRS | Homo sapiens (human) |
| Maleylacetoacetate isomerase | MAAI; EC 5.2.1.2; GSTZ1-1; Glutathione S-transferase zeta 1; 2.5.1.18 | Homo sapiens (human) |
| Phenylalanine--tRNA ligase beta subunit | EC 6.1.1.20; Phenylalanyl-tRNA synthetase beta subunit; PheRS | Homo sapiens (human) |
| Tyrosine aminotransferase | TAT; EC 2.6.1.5; L-tyrosine:2-oxoglutarate aminotransferase | Homo sapiens (human) |
| Aspartate aminotransferase, cytoplasmic | cAspAT; EC 2.6.1.1; EC 2.6.1.3; Cysteine aminotransferase, cytoplasmic; Cysteine transaminase, cytoplasmic; cCAT; Glutamate oxaloacetate transaminase 1; Transaminase A | Homo sapiens (human) |
| Phenylalanine-4-hydroxylase | PAH; EC 1.14.16.1; Phe-4-monooxygenase | Homo sapiens (human) |
| Phenylalanine--tRNA ligase alpha subunit | EC 6.1.1.20; CML33; Phenylalanyl-tRNA synthetase alpha subunit; PheRS | Homo sapiens (human) |
| 4-hydroxyphenylpyruvate dioxygenase | EC 1.13.11.27; 4-hydroxyphenylpyruvic acid oxidase; 4HPPD; HPD; HPPDase | Homo sapiens (human) |
| L-amino-acid oxidase | LAAO; LAO; EC 1.4.3.2; EC 1.4.3.25; Interleukin-4-induced protein 1; IL4-induced protein 1; hIL4I1; Protein Fig-1; hFIG1 | Homo sapiens (human) |
| Homogentisate 1,2-dioxygenase | EC 1.13.11.5; Homogentisate oxygenase; Homogentisic acid oxidase; Homogentisicase | Homo sapiens (human) |
| Fumarylacetoacetase | FAA; EC 3.7.1.2; Beta-diketonase; Fumarylacetoacetate hydrolase | Homo sapiens (human) |
Compounds (17)
| Compound | Description |
|---|
| ammonium hydroxide | The hydroxy salt of ammonium ion. It is formed when AMMONIA reacts with water molecules in solution. |
| Carbon Dioxide | A colorless, odorless gas that can be formed by the body and is necessary for the respiration cycle of plants and animals. |
| glutamic acid | A non-essential amino acid naturally occurring in the L-form. Glutamic acid is the most common excitatory neurotransmitter in the CENTRAL NERVOUS SYSTEM. |
| Adenosine Triphosphate | An adenine nucleotide containing three phosphate groups esterified to the sugar moiety. In addition to its crucial roles in metabolism adenosine triphosphate is a neurotransmitter. |
| tyrosine | A non-essential amino acid. In animals it is synthesized from PHENYLALANINE. It is also the precursor of EPINEPHRINE; THYROID HORMONES; and melanin. |
| adenosine monophosphate | Adenine nucleotide containing one phosphate group esterified to the sugar moiety in the 2'-, 3'-, or 5'-position. |
| phenylalanine | An essential aromatic amino acid that is a precursor of MELANIN; DOPAMINE; noradrenalin (NOREPINEPHRINE), and THYROXINE. |
| Hydrogen Peroxide | A strong oxidizing agent used in aqueous solution as a ripening agent, bleach, and topical anti-infective. It is relatively unstable and solutions deteriorate over time unless stabilized by the addition of acetanilide or similar organic materials. |
| Water | A clear, odorless, tasteless liquid that is essential for most animal and plant life and is an excellent solvent for many substances. The chemical formula is hydrogen oxide (H2O). (McGraw-Hill Dictionary of Scientific and Technical Terms, 4th ed) |
| Oxygen | An element with atomic symbol O, atomic number 8, and atomic weight [15.99903; 15.99977]. It is the most abundant element on earth and essential for respiration. |
| alpha-ketoglutaric acid | |
| fumarylacetoacetate | metabolite of tyrosine; RN given for (E)-isomer; structure in first source |
| homogentisic acid | Dihydroxyphenylacetic acid with hydroxyls at the 2 and 5 positions of the phenyl ring. |
| acetoacetic acid | |
| pyrophosphate | |
| 4-hydroxyphenylpyruvic acid | RN given refers to parent cpd |
| phenylpyruvic acid | RN given refers to parent cpd |