TH
GeneName
TH
Summary
Tyrosine hydroxylase (TH), also known as tyrosine 3 monooxygenase, is a 59 kDa enzyme predominantly expressed in neurons, particularly in dopaminergic pathways. It is located in various cellular compartments including the cytoplasm, nucleus, and synaptic vesicles. TH catalyses the conversion of tyrosine to L-DOPA, the precursor of dopamine, norepinephrine, and epinephrine, playing a crucial role in catecholamine biosynthesis. This enzyme is essential for the regulation of neurotransmitter levels and is involved in several physiological processes such as locomotion, learning, and memory.
Importance
TH is relevant to: - Neurodegenerative diseases, particularly Parkinson's disease, due to its role in dopamine synthesis and neurotransmission - Psychiatric disorders linked to dopamine dysregulation, including schizophrenia and depression - Cardiovascular research, as it influences norepinephrine levels and heart function - Developmental biology, given its involvement in organ morphogenesis and eye development
Top Products
For researchers investigating TH, we recommend two excellent primary antibodies. The first is the well-cited polyclonal antibody, Anti-Tyrosine Hydroxylase antibody - Neuronal Marker (ab112), which has garnered 485 citations, reflecting its strong reputation in the field. This antibody is particularly effective for immunohistochemistry (IHC) and western blotting (WB). In addition, we offer the recombinant antibody, Anti-Tyrosine Hydroxylase antibody [EP1532Y] - Neuronal Marker (ab137869). This product has been validated for use in IHC, WB, and immunocytochemistry (ICC), making it a versatile choice for various applications. With 132 citations, it is also gaining traction among researchers. The recombinant nature of this antibody ensures batch-to-batch consistency, which is essential for reproducible results in your experiments. The Anti-Tyrosine Hydroxylase antibody - Neuronal Marker ELISA Kit (ab76442), with 236 citations, is an excellent option for researchers looking to accurately measure TH levels in neuronal samples.
Abcam Product Citation Summary
The data indicates a significant focus on the use of Abcam antibodies for detecting Tyrosine Hydroxylase (TH) in various species, particularly in mouse and rat models. The applications primarily involve Western Blotting (WB) and Immunohistochemistry (IHC), highlighting the relevance of TH in studies related to neurobiology, including neuronal activity, neuroprotection, and the effects of various treatments on dopaminergic systems.
Abcam Product Citation Table
Function
Catalyzes the conversion of L-tyrosine to L-dihydroxyphenylalanine (L-Dopa), the rate-limiting step in the biosynthesis of catecholamines, dopamine, noradrenaline, and adrenaline. Uses tetrahydrobiopterin and molecular oxygen to convert tyrosine to L-Dopa (PubMed:15287903, PubMed:1680128, PubMed:17391063, PubMed:24753243, PubMed:34922205, PubMed:8528210, Ref.18). 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.
Involvement in disease
Segawa syndrome autosomal recessive
ARSEGS
A form of DOPA-responsive dystonia presenting in infancy or early childhood. Dystonia is defined by the presence of sustained involuntary muscle contractions, often leading to abnormal postures. Some cases present with parkinsonian symptoms in infancy. Unlike all other forms of dystonia, it is an eminently treatable condition, due to a favorable response to L-DOPA.
None
The disease is caused by variants affecting the gene represented in this entry.
May play a role in the pathogenesis of Parkinson disease (PD). A genome-wide copy number variation analysis has identified a 34 kilobase deletion over the TH gene in a PD patient but not in any controls.
Pathway
Catecholamine biosynthesis; dopamine biosynthesis; dopamine from L-tyrosine: step 1/2.
Post-translational modifications
Phosphorylated on Ser-19, Ser-62 and Ser-71 by several protein kinases with different site specificities. Phosphorylation at Ser-62 and Ser-71 leads to an increase of TH activity (PubMed:7901013). Phosphorylation at Ser-71 activates the enzyme and also counteracts the feedback inhibition of TH by catecholamines (PubMed:15287903). Phosphorylation of Ser-19 and Ser-62 triggers the proteasomal degradation of TH through the ubiquitin-proteasome pathway (By similarity). Phosphorylation at Ser-62 facilitates transport of TH from the soma to the nerve terminals via the microtubule network (PubMed:28637871). Phosphorylation at Ser-19 induces the high-affinity binding to the 14-3-3 protein YWHAG; this interaction may influence the phosphorylation and dephosphorylation of other sites (PubMed:24947669). Ser-19 increases the phosphorylation at Ser-71 in a hierarchical manner, leading to increased activity (By similarity).
Sequence Similarities
Belongs to the biopterin-dependent aromatic amino acid hydroxylase family.
Tissue Specificity
Mainly expressed in the brain and adrenal glands.
Cellular localization
- Cytoplasm
- Perinuclear region
- Nucleus
- Cell projection
- Axon
- Cytoplasm
- Cytoplasmic vesicle
- Secretory vesicle
- Synaptic vesicle
- When phosphorylated at Ser-19 shows a nuclear distribution and when phosphorylated at Ser-31 as well at Ser-40 shows a cytosolic distribution (By similarity). Expressed in dopaminergic axons and axon terminals.
Alternative names
TYH, TH, Tyrosine 3-monooxygenase, Tyrosine 3-hydroxylase