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	OMIM : 191290 ; UniProt : P07101 ;
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

+ BH4 + O ₂ + BH4-OH ${\ displaystyle \ longrightarrow}$

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

[1] UniProt P07101

[2] 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 .

[3] 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 .