Tyrosine hydroxylase
Tyrosine hydroxylase | ||
---|---|---|
Model of the TH according to PDB 2XSN | ||
Properties of human protein | ||
Mass / length primary structure | 527 amino acids | |
Secondary to quaternary structure | Homotetramer | |
Cofactor | Iron (II), tetrahydrobiopterin | |
Isoforms | 4th | |
Identifier | ||
Gene names | TH , TYH | |
External IDs | ||
Enzyme classification | ||
EC, category | 1.14.16.2 , monooxygenase | |
Response type | Hydroxylation | |
Substrate | L-tyrosine + tetrahydrobiopterin + O 2 | |
Products | 3,4-dihydroxy-L-phenylalanine + 4a-hydroxytetrahydrobiopterine |
The tyrosine hydroxylase (TYH) is that enzyme which catalyzes the conversion of the amino acid L- tyrosine in the amino acid levodopa catalyzes the rate limiting reaction step in the biosynthesis of catecholamines . TYH is found in multicellular animals . In humans, it is produced in the adrenal glands and the brain . Mutations in TH - gene can enzyme deficiency and (rare) Segawa syndrome lead. TYH is an autoantigen in the disease APECED .
The TYH is inhibited by the docking of dopamine at two points on the enzyme. TYH can be reactivated by phosphorylation of a serine residue at one of the docking sites.
The tyrosine hydroxylase uses tetrahydrobiopterin as a cofactor.
Catalyzed reaction
L-tyrosine is oxidized to L-DOPA.
Individual evidence
- ↑ UniProt P07101
- ↑ Hedstrand H, O Ekwall, Haavik J, et al : Identification of tyrosine hydroxylase as to autoantigen in autoimmune syndrome type I polyendocrine . In: Biochem. Biophys. Res. Commun. . 267, No. 1, January 2000, pp. 456-61. doi : 10.1006 / bbrc.1999.1945 . PMID 10623641 .
- ^ Gordon SL, Quinsey NS, Dunkley PR, Dickson PW: Tyrosine hydroxylase activity is regulated by two distinct dopamine-binding sites . In: J. Neurochem. . 106, No. 4, August 2008, pp. 1614-23. doi : 10.1111 / j.1471-4159.2008.05509.x . PMID 18513370 .
Web links
Wikibooks: Biochemistry and Pathobiochemistry: Tyrosine Metabolism - Learning and Teaching Materials
Wikibooks: Biochemistry and Pathobiochemistry: Biopterin Metabolism - Learning and Teaching Materials
- Jassal / D'Eustachio / reactome.org: Tyrosine is hydroxylated to dopa