Tyrosine 3-hydroxylase

TH
Available structures PDB Ortholog search: PDBe RCSB List of PDB id codes 2XSN, 4J6S
Identifiers
Aliases	TH, Th, DYT14, DYT5b, TYH, tyrosine hydroxylase, Tyrosine hydroxylase
External IDs	MGI: 98735 HomoloGene: 307 GeneCards: TH
Gene location (Human) Chr. Chromosome 11 (human) Band 11p15.5 Start 2,163,929 bp End 2,171,877 bp
Gene location (Mouse) Chr. Chromosome 7 (mouse) Band 7 F5|7 88.06 cM Start 142,892,752 bp End 142,931,128 bp
RNA expression pattern More reference expression data
Gene ontology Molecular function • protein domain specific binding • tetrahydrobiopterin binding • tyrosine 3-monooxygenase activity • oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen, reduced pteridine as one donor, and incorporation of one atom of oxygen • ferrous iron binding • oxygen binding • metal ion binding • dopamine binding • enzyme binding • oxidoreductase activity • iron ion binding • ferric iron binding • protein binding • amino acid binding • monooxygenase activity Cellular component • cytoplasm • cytosol • mitochondrion • neuron projection • cytoplasmic side of plasma membrane • nucleus • synaptic vesicle • melanosome membrane • terminal bouton • perikaryon • axon • neuronal cell body • smooth endoplasmic reticulum • dendrite • cytoplasmic vesicle membrane • cytoplasmic vesicle Biological process • regulation of heart contraction • response to salt stress • response to steroid hormone • response to amphetamine • isoquinoline alkaloid metabolic process • response to zinc ion • terpene metabolic process • learning • epinephrine biosynthetic process • synaptic transmission, dopaminergic • circadian sleep/wake cycle • cellular response to drug • multicellular organism aging • animal organ morphogenesis • response to growth factor • response to ethanol • cellular response to glucose stimulus • phthalate metabolic process • locomotory behavior • response to light stimulus • anatomical structure morphogenesis • response to nicotine • response to metal ion • memory • heart development • response to nutrient levels • embryonic camera-type eye morphogenesis • social behavior • cellular response to manganese ion • response to ether • sphingolipid metabolic process • response to immobilization stress • norepinephrine biosynthetic process • cognition • dopamine biosynthetic process • response to corticosterone • cellular response to nicotine • cellular response to alkaloid • mating behavior • response to electrical stimulus • response to isolation stress • glycoside metabolic process • aromatic amino acid family metabolic process • response to lipopolysaccharide • cerebral cortex development • response to pyrethroid • pigmentation • phytoalexin metabolic process • response to herbicide • visual perception • response to estradiol • response to hypoxia • response to organic cyclic compound • eating behavior • cellular response to growth factor stimulus • heart morphogenesis • response to water deprivation • catecholamine biosynthetic process • neurotransmitter biosynthetic process • response to peptide hormone • sensory perception of sound • response to activity • fatty acid metabolic process • eye photoreceptor cell development • response to insecticide • dopamine biosynthetic process from tyrosine • oxidation-reduction process • aminergic neurotransmitter loading into synaptic vesicle • response to drug Sources:Amigo / QuickGO
Orthologs
Species	Human	Mouse
Entrez	7054	21823
Ensembl	ENSG00000180176	ENSMUSG00000000214
UniProt	P07101 P78428	P24529
RefSeq (mRNA)	NM_000360 NM_199292 NM_199293	NM_009377
RefSeq (protein)	NP_000351 NP_954986 NP_954987 NP_954986.2 NP_954987.2	NP_033403
Location (UCSC)	Chr 11: 2.16 – 2.17 Mb	Chr 11: 142.89 – 142.93 Mb
PubMed search

tyrosine 3-monooxygenase
Tyrosine hydroxylase catalyzes conversion of tyrosine to L-DOPA using Fe2+, O2 and BH4
Identifiers
EC number	1.14.16.2
CAS number	9036-22-0
Databases
IntEnz	IntEnz view
BRENDA	BRENDA entry
ExPASy	NiceZyme view
KEGG	KEGG entry
MetaCyc	metabolic pathway
PRIAM	profile
PDB structures	RCSB PDB PDBe PDBsum
Gene Ontology	AmiGO / QuickGO
Search PMC articles PubMed articles NCBI proteins

2XSN, 4J6S

7054

21823

ENSG00000180176

ENSMUSG00000000214

P07101
P78428

P24529

NM_000360
NM_199292
NM_199293

NM_009377

NP_000351
NP_954986
NP_954987
NP_954986.2
NP_954987.2

NP_033403

Tyrosine hydroxylase or tyrosine 3-monooxygenase is the enzyme responsible for catalyzing the conversion of the amino acid L-tyrosine to L-3,4-dihydroxyphenylalanine (L-DOPA). It does so using molecular oxygen (O₂), as well as iron (Fe²⁺) and tetrahydrobiopterin as cofactors. L-DOPA is a precursor for dopamine, which, in turn, is a precursor for the important neurotransmitters norepinephrine (noradrenaline) and epinephrine (adrenaline). Tyrosine hydroxylase catalyzes the rate limiting step in this synthesis of catecholamines. In humans, tyrosine hydroxylase is encoded by the TH gene, and the enzyme is present in the central nervous system (CNS), peripheral sympathetic neurons and the adrenal medulla. Tyrosine hydroxylase, phenylalanine hydroxylase and tryptophan hydroxylase together make up the family of aromatic amino acid hydroxylases (AAAHs).