Gene and Protein Information
Species	TM	AA	Chromosomal Location	Gene Symbol	Gene Name	Reference
Human	-	528	11p15.5	TH	tyrosine hydroxylase
Mouse	-	498	7 88.06 cM	Th	tyrosine hydroxylase
Rat	-	498	1q41	Th	tyrosine hydroxylase

Previous and Unofficial Names

Tyrosine 3-monooxygenase | The | tyrosine 3-hydroxylase

Database Links
Alphafold	P07101 (Hs), P24529 (Mm), P04177 (Rn)
BRENDA	1.14.16.2
CATH/Gene3D	1.10.800.10
ChEMBL Target	CHEMBL1969 (Hs), CHEMBL2462 (Rn)
DrugBank Target	P07101 (Hs)
Ensembl Gene	ENSG00000180176 (Hs), ENSMUSG00000000214 (Mm), ENSRNOG00000020410 (Rn)
Entrez Gene	7054 (Hs), 21823 (Mm), 25085 (Rn)
Human Protein Atlas	ENSG00000180176 (Hs)
KEGG Enzyme	1.14.16.2
KEGG Gene	hsa:7054 (Hs), mmu:21823 (Mm), rno:25085 (Rn)
OMIM	191290 (Hs)
Pharos	P07101 (Hs)
UniProtKB	P07101 (Hs), P24529 (Mm), P04177 (Rn)
Wikipedia	TH (Hs)

Enzyme Reaction
EC Number: 1.14.16.2 L-tyrosine + O2 -> levodopa

Substrates and Reaction Kinetics
Substrate Sp. Property Value Units Standard property Standard value Assay description Assay conditions Comments Reference L-tyrosine N/A - -

Products
Product Species Reference levodopa None

Cofactors
Cofactor Species Comments Reference sapropterin Human Fe2+ Human

Endogenous activators

Protein kinase A-mediated phosphorylation  [1]

Download all structure-activity data for this target as a CSV file

Inhibitors
Key to terms and symbols	View all chemical structures	Click column headers to sort
Ligand Sp. Action Value Parameter Reference methyltyrosine Hs Inhibition - - ⤷ α-propyldopacetamide Hs Inhibition - - ⤷ 3-chlorotyrosine Hs Inhibition - - ⤷ 3-iodotyrosine Hs Inhibition - - ⤷ alpha-methyltyrosine Hs Inhibition - - ⤷

Clinically-Relevant Mutations and Pathophysiology
Disease:  Segawa syndrome, autosomal recessive Synonyms: Autosomal recessive dopa-responsive dystonia [Orphanet: ORPHA101150] OMIM: 605407 Orphanet: ORPHA101150