Tyrosine 3-monooxygenase

Definition:

Catalyzes the conversion of L-tyrosine to L-dihydroxyphenylalanine (L-Dopa), the rate-limiting step in the biosynthesis of cathecolamines, dopamine, noradrenaline, and adrenaline. Uses tetrahydrobiopterin and molecular oxygen to convert tyrosine to L-Dopa. In addition to tyrosine, is able to catalyze the hydroxylation of phenylalanine and tryptophan with lower specificity (By similarity). Positively regulates the regression of retinal hyaloid vessels during postnatal development (By similarity).; [Isoform 5]: Lacks catalytic activity.; [Isoform 6]: Lacks catalytic activity.

References:

[1]. Shunya Inukai, et al. Tyrosine hydroxylase activity is regulated through the modification of the 176th cysteine residue. Biochem Biophys Res Commun. 2022 Jan 22;589:209-214. [Content Brief]

[2]. Giri R Sura, et al. Effects of phosphorylation by protein kinase A on binding of catecholamines to the human tyrosine hydroxylase isoforms. J Neurochem. 2004 Aug;90(4):970-8. [Content Brief]

[3]. Josep Roma, et al. Characterisation of novel splicing variants of the tyrosine hydroxylase C-terminal domain in human neuroblastic tumours. Biol Chem. 2007 Apr;388(4):419-26. [Content Brief]

[4]. P M Knappskog, et al. Recessively inherited L-DOPA-responsive dystonia caused by a point mutation (Q381K) in the tyrosine hydroxylase gene. Hum Mol Genet. 1995 Jul;4(7):1209-12. [Content Brief]

[5]. Agnete Fossbakk, et al. Functional studies of tyrosine hydroxylase missense variants reveal distinct patterns of molecular defects in Dopa-responsive dystonia. Hum Mutat. 2014 Jul;35(7):880-90. [Content Brief]

[6]. B Le Bourdellès, et al. Phosphorylation of human recombinant tyrosine hydroxylase isoforms 1 and 2: an additional phosphorylated residue in isoform 2, generated through alternative splicing. J Biol Chem. 1991 Sep 15;266(26):17124-30. [Content Brief]

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