Tyrosine hydroxylase deficiency (THD) is a disorder caused by disfunction of tyrosine hydroxylase, an enzyme involved in the biosynthesis of dopamine. This condition is one of the causes of dopa-responsive dystonia.[1]
^Weissbach A, Pauly MG, Herzog R, Hahn L, Halmans S, Hamami F, Bolte C, Camargos S, Jeon B, Kurian MA, Opladen T, Brüggemann N, Huppertz HJ, König IR, Klein C, Lohmann K (February 2022). "Relationship of Genotype, Phenotype, and Treatment in Dopa-Responsive Dystonia: MDSGene Review". Movement Disorders. 37 (2): 237–252. doi:10.1002/mds.28874. PMID 34908184. S2CID 245260405.
and 23 Related for: Tyrosine hydroxylase deficiency information
Tyrosinehydroxylase or tyrosine 3-monooxygenase is the enzyme responsible for catalyzing the conversion of the amino acid L-tyrosine to L-3,4-dihydroxyphenylalanine...
Phenylalanine hydroxylase (PAH) (EC 1.14.16.1) is an enzyme that catalyzes the hydroxylation of the aromatic side-chain of phenylalanine to generate tyrosine. PAH...
contains phenylalanine. The amino acid tyrosine becomes essential in people with phenylalanine hydroxylasedeficiency. Thus, in addition to the careful reduction...
doi:10.1146/annurev-arplant-042811-105439. PMID 22554242. "Tyrosinehydroxylasedeficiency". Genetics Home Reference. U.S. National Library of Medicine...
fight-or-flight response. Tyrosine is created from phenylalanine by hydroxylation by the enzyme phenylalanine hydroxylase. Tyrosine is also ingested directly...
tyrosine, another amino acid, by phenylalanine hydroxylase. However, this enzyme requires tetrahydrobiopterin as a cofactor and thus its deficiency slows...
(5-HTP). Phenylalanine hydroxylase (PAH) catalyses the conversion of L-phenylalanine (PHE) to L-tyrosine (TYR). Therefore, a deficiency in tetrahydrobiopterin...
into dopamine. 17α-Hydroxylase Cholesterol 7 alpha-hydroxylase Dopamine β-hydroxylase Phenylalanine hydroxylaseTyrosinehydroxylase One example of non-biological...
prolyl hydroxylase and lysyl hydroxylase, both requiring vitamin C as a cofactor. The role of vitamin C as a cofactor is to oxidize prolyl hydroxylase and...
activated T and B cells. In vitro, vitamin D increases expression of the tyrosinehydroxylase gene in adrenal medullary cells, and affects the synthesis of neurotrophic...
Rakovich, Tatsiana; et al. (12 April 2011). "Queuosine deficiency in eukaryotes compromises tyrosine production through increased tetrahydrobiopterin oxidation"...
(melanogenesis) starts with the oxidation of l-tyrosine to l-dopa by the enzyme tyrosinehydroxylase, then to l-dopaquinone and dopachrome, which forms...
terminal. This inhibits the synthesis of norepinephrine by inhibiting tyrosinehydroxylase. The S-enantiomer of methyldopa is a competitive inhibitor of the...
studies. The group studies deficiencies in aromatic amino acid decarboxylase, tyrosinehydroxylase, dopamine beta-hydroxylase, monoamine oxidase A, dopamine...
cannot be stimulated by circulating epinephrine . In dopamine beta hydroxylasedeficiency, the entire body cannot efficiently produce epinephrine and norepinephrine...
impairment, is comparable with autosomal dominant GTPCH and tyrosinehydroxylasedeficiency, which are both classified as forms of DOPA-responsive dystonia...
Autosomal dominant GTP cyclohydrolase I deficiency (AD-GTPCHD) is a disease caused by dysfunction of GTP cyclohydrolase I, an enzyme that plays an important...
tyrosine, a non-essential amino acid derived from food or produced from phenylalanine in the liver. The enzyme tyrosinehydroxylase converts tyrosine...
Postorgasmic illness syndrome (POIS), through production of tyrosinehydroxylase and dopamine β-hydroxylase, which two enzymes comprise the biochemical mechanism...
to convert phenylalanine to tyrosine as a result of the entire or partial absence of the enzyme phenylalanine hydroxylase. The coloration of the skin...
metabolic disorder affecting the metabolism of tyrosine. Normally, the breakdown of the amino acid tyrosine involves the conversion of 4-hydroxyphenylpyruvate...
converted into L-tyrosine by the enzyme phenylalanine hydroxylase, with molecular oxygen (O2) and tetrahydrobiopterin as cofactors. L-Tyrosine is converted...