Tyrosine Hydroxylase Products

Description

The tetrameric enzyme tyrosine hydroxylase (TH), also designated as tyrosine 3-monooxygenase (TY3H), is the rate-limiting enzyme for catecholamine biosynthesis and has a theoretical molecular weight of 60 kDa. Encoded by the TH gene, TH catalyzes the conversion of the amino acid L-tyrosine to L-3,4- dihydroxyphenylalanine (L-dopa) in the central nervous system and adrenal medulla. The precursor of catecholamines, L-dopa, is converted to dopamine for use in the biosynthesis of neurotransmitters norepinephrine (noradrenaline) and epinephrine (adrenaline) (1) . Dysfunction in the TH gene, and thus the enzymatic activity by TH, dysregulates catecholamine synthesis.

Two transcription factor binding sites in the proximal region of the TH gene, the TPA-responsive element (TRE) and the c-AMP responsive element (CRE), have been implicated in the complex regulation of the TH gene. Dysregulation of breakdown for the amino acid, tyrosine, by TH is a result of a genetic disorder that results in Tyrosinemia (high levels of tyrosine in the blood, tissue and organs).

Tyrosine hydroxylase deficiency is a disorder that primarily affects movement, where individuals display symptoms that include lack of coordination when walking, postural tremors and unusual body positioning. TH deficient dopamine-responsive dystonia (DRD), also known as Segawa syndrome, is a rare genetic disorder that is associated with low levels of TH and is diagnosed during childhood with characteristic symptoms including increased muscle tone (dystonia) and signs of Parkinsonism like bradykinesia, tremors, rigidity and postural instability (2). Correspondingly, TH is also linked to Parkinson's disease in older adults, where low dopamine levels are a consistent neurochemical abnormality. Functional polymorphisms of the TH gene may be involved in the pathogenesis of neuropsychiatric diseases such as schizophrenia and other affective disorders where dopamine is often dysregulated (3).

References

1. Hamanaka, Y., & Mizunami, M. (2019). Tyrosine hydroxylase-immunoreactive neurons in the mushroom body of the field cricket, Gryllus bimaculatus. Cell Tissue Res, 376(1), 97-111. doi:10.1007/s00441-018-2969-9

2. Li, L., & Zhou, F. M. (2013). Parallel dopamine D1 receptor activity dependence of l-Dopa-induced normal movement and dyskinesia in mice. Neuroscience, 236, 66-76. doi:10.1016/j.neuroscience.2012.12.065

3. Borkar, C. D., Bharne, A. P., Nagalakshmi, B., Sakharkar, A. J., Subhedar, N. K., & Kokare, D. M. (2018). Cocaine- and Amphetamine-Regulated Transcript Peptide (CART) Alleviates MK-801-Induced Schizophrenic Dementia-Like Symptoms. Neuroscience, 375, 94-107. doi:10.1016/j.neuroscience.2018.01.056

Bioinformatics

Entrez	21823 Mouse 25085 Rat 7054 Human
Uniprot	NP_000351 Human NP_954986.2 Human P04177 Rat P07101 Human P07101-2 Human P24529 Mouse
Product By Gene ID	7054
Alternate Names	DYT14 DYT5b EC 1.14.16 EC 1.14.16.2 TH TYH dystonia 14 Tyrosine 3-hydroxylase tyrosine 3-monooxygenase tyrosine hydroxylase

Research Areas for Tyrosine Hydroxylase

Find related products by research area and learn more about each of the different research areas below.

Lipid and Metabolism
Neuronal Cell Markers
Neuroscience
Neurotransmission
Phospho-Specific
Prostate Cancer
Tyrosine Kinases

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Tyrosine hydroxylase - a marker for dopaminergic neurons in the central nervous system Tyrosine hydroxylase is a member of the aromatic amino acid hydroxylase (AAAH) family.  It is expressed throughout the central nervous system (CNS) and catalyzes the conversion of tyrosine to L-3,4-dihydroxyphenylalanine (L-DOPA), which can be, thr...    Read more.

The identification of dopaminergic neurons using Tyrosine Hydroxylase in Parkinson's research and LRRK2 Tyrosine hydroxylase (TH) is a crucial enzyme involved in the biosynthesis of dopamine, norepinephrine and epinephrine in the brain.  Specifically, TH catalyzes the conversion of l-tyrosine to l-dihydroxyphenylalanine (l-dopa).  The importance of t...    Read more.

Tyrosine Hydroxylase Deficiency and Brain Disorders  Tyrosine hydroxylase catalyzes the rate-limiting step in the biosynthesis of the catecholamines dopamine, norepinephrine, and epinephrine. A hallmark of Parkinson's disease is the loss of dopaminergic neurons in the substantia nigra. Mutations in cas...    Read more.

Tyrosine Hydroxylase Deficiencies and Neurodegeneration Tyrosine hydroxylase is the rate-limiting enzyme in the synthesis pathway of the catecholamines dopamine, epinephrine, and norepinephrine. Alternative mRNA splicing generates a wide molecular diversity of TH isoforms that are tissue specific and produ...    Read more.

A Big Guy for the Catecholamine Synthesis - Tyrosine hydroxylase (TH) In the synthesis pathway for the catecholamines - dopamine, epinephrine, and norepinephrine, tyrosine hydroxylase is the rate-limiting enzyme. Through alternative mRNA splicing, a wide molecular diversity of TH isoforms are generated that are tissue-s...    Read more.

Successful Transplantation of Friedreich Ataxia Induced Pluripotent Stem Cell (iPSC)-Derived Sensory Neurons in Dorsal Root Ganglia of Adult Rodents Jamshed Arslan, Pharm D, PhD The dorsal root ganglia (DRG) are a collection of cell bodies of sensory nerves carrying sensory information – including nociception, mechanoreception and proprioception – from periphera...    Read more.

Tyrosine Hydroxylase - rate-limiting enzyme in catecholamine synthesis Catecholamines are tyrosine-derived hormones that are produced in the adrenal gland. They include epinephrine, norepinephrine, and dopamine and are used as neurotransmitters by the central and peripheral nervous system. The rate limiting enzyme in ...    Read more.

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