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TH / Tyrosine Hydroxylase Protein, Antibody, ELISA Kit, cDNA Clone

TH / Tyrosine Hydroxylase Related Areas

TH / Tyrosine Hydroxylase Related Pathways

TH / Tyrosine Hydroxylase Related Product

    TH / Tyrosine Hydroxylase Summary & Protein Information

    TH / Tyrosine Hydroxylase Background

    Gene Summary: The protein encoded by TH gene is involved in the conversion of tyrosine to dopamine. It is the rate-limiting enzyme in the synthesis of catecholamines, hence plays a key role in the physiology of adrenergic neurons. Mutations in TH gene have been associated with autosomal recessive Segawa syndrome. Alternatively spliced transcript variants encoding different isoforms have been noted for TH gene. [provided by RefSeq, Jul 2008]
    General information above from NCBI
    Catalytic activity: L-tyrosine + tetrahydrobiopterin + O(2) = L- dopa + 4a-hydroxytetrahydrobiopterin.
    Cofactor: Fe(2+) ion.
    Enzyme regulation: Phosphorylation leads to an increase in the catalytic activity.
    Tissue specificity: Mainly expressed in the brain and adrenal glands.
    Involvement in disease: Segawa syndrome autosomal recessive (ARSEGS) [MIM:605407]: 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. Note=The disease is caused by mutations affecting the gene represented in this entry.
    Note=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.
    Sequence similarity: Belongs to the biopterin-dependent aromatic amino acid hydroxylase family.
    General information above from UniProt

    Tyrosine hydroxylase (TH) is a rate-limiting enzyme in catecholamine synthesis. Tyrosine hydroxylase activity is modulated by protein-protein interactions with enzymes in the same pathway or the tetrahydrobiopterin pathway, structural proteins considered to be chaperones that mediate the neuron's oxidative state. It is phosphorylated at serine (Ser) residues Ser8, Ser19, Ser31 and Ser40 in vitro. The phosphorylation of tyrosine hydroxylase at Ser19 or Ser8 has no direct effect on tyrosine hydroxylase activity. As tyrosine hydroxylase (TH) catalyses the formation of L-DOPA, the rate-limiting step in the biosynthesis of DA, the Parkinson's disease (PD) can be considered as a TH-deficiency syndrome of the striatum. A direct pathogenetic role of TH has also been suggested, as the enzyme is a source of reactive oxygen species (ROS) in vitro and a target for radical-mediated oxidative injury. Recently, it has been demonstrated that L-DOPA is effectively oxidized by mammalian Tyrosine hydroxylase in vitro, possibly contributing to the cytotoxic effects of DOPA.

    TH / Tyrosine Hydroxylase Alternative Name

    TH / Tyrosine Hydroxylase Related Studies

  • Daubner SC, et al. (2011) Tyrosine hydroxylase and regulation of dopamine synthesis. Arch Biochem Biophys. 508(1): 1-12.
  • Dunkley PR, et al. (2004) Tyrosine hydroxylase phosphorylation: regulation and consequences. J Neurochem. 91(5): 1025-43.
  • Haavik J, et al. (1998) Tyrosine hydroxylase and Parkinson's disease. Mol Neurobiol. 16(3): 285-309.
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