| Gene Symbol | PAHPROTEIN |
| Protein Type | Enzyme |
| Function | is a crucial enzyme in phenylalanine metabolism, catalyzing the rate-limiting step in the catabolic pathway that converts the essential amino acid L-phenylalanine to L-tyrosine [blau2010](https://p... |
| Primary Expression | the liver, where it functions as a homotetramer to metabolize the majority of dietary phenylalanine |
| Molecular Weight | 52 kDa |
| Amino Acids | 507 aa |
| GeneCards | PAHPROTEIN |
| Human Protein Atlas | PAHPROTEIN |
| Regulatory Mechanism | Effect on PAH Activity |
| Phenylalanine concentration | Allosteric activation at high [Phe] |
| Phosphorylation (Ser16) | Increases specific activity |
| BH₄ availability | Absolute requirement for catalysis |
| Hepatic phenylalanine levels | Diurnal variation in activity |
| Associated Diseases | ALS, Aging, Alzheimer, Atherosclerosis, Autism |
| Interactions | LDLR, GFAP, LRP4, LRP8, LRP5, LRP1 |
| KG Connections | 207 knowledge graph edges |
| Databases | GeneCardsUniProtNCBI GeneHPASTRING |