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

More general terms

• hydroxylase; monooxygenase
• phosphoprotein

Additional terms

• metyrosine (Demser)

Database

• UniProt: http://www.uniprot.org/uniprot/P07101.html
• OMIM: https://mirror.omim.org/entry/191290
• OMIM: https://mirror.omim.org/entry/605407