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tyrosine 3-hydroxylase (tyrosine 3-monooxygenase, TH, TYH)
Function:
- role in the physiology of adrenergic neurons
- phosphorylation increases catalytic activity
- catecholamine biosynthesis, 1st step
L-tyrosine + tetrahydrobiopterin + O2
3,4-dihydroxy-L-phenylalanine + 4a-hydroxytetrahydrobiopterin
Cofactor: Fe+2 ion
Structure:
- belongs to the biopterin-dependent aromatic amino acid hydroxylase family
Alternative splicing: named isoforms=4
Expression: mainly expressed in the brain & adrenal glands
Pathology:
- defects in TH are the cause of autosomal recessive Segawa syndrome
Interactions
molecular events
Related
metyrosine (Demser)
General
hydroxylase; monooxygenase
phosphoprotein
Properties
SIZE: entity length = 528 aa
MW = 59 kD
MOTIF: Ser phosphorylation site {S19}
Ser phosphorylation site {S62}
Ser phosphorylation site {S71}
alanine-rich region {85-90}
MOTIF: alanine residue (SEVERAL)
Iron [Fe]-binding site
SITE: 361-361
Iron [Fe]-binding site
SITE: 366-366
Iron [Fe]-binding site
SITE: 406-406
Database Correlations
OMIM correlations
MORBIDMAP 191290
UniProt P07101
Pfam PF00351
KEGG correlations
ENZYME 1.14.16.2
References
- UniProt :accession P07101
- GeneReviews
https://www.genecards.org/cgi-bin/carddisp.pl?gene=TH
- Wikipedia; Tyrosine hydroxylase entry
http://en.wikipedia.org/wiki/Tyrosine_hydroxylase