Kaufman S (1962). "Phenylalanine hydroxylase". Methods Enzymol. 5: 809-816. doi:10.1016/s0076-6879(62)05317-3. Lind KE ( ...
Mitchell, J. J.; Trakadis, Y. J.; Scriver, C. R. (2011). "Phenylalanine hydroxylase deficiency". Genetics in Medicine. 13 (8): ... Robert Guthrie developed a simple method using a bacterial inhibition assay that could detect high levels of phenylalanine in ... is caused by a deficiency of the enzyme steroid 21-hydroxylase, and comes in two forms - simple virilizing and a salt-wasting ... a metabolic condition in which the inability to degrade the essential amino acid phenylalanine can cause irreversible mental ...
Mitchell, J. J.; Trakadis, Y. J.; Scriver, C. R. (2011). "Phenylalanine hydroxylase deficiency". Genetics in Medicine. 13 (8): ...
The enzyme phenylalanine hydroxylase normally converts the amino acid phenylalanine into the amino acid tyrosine. If this ... are caused by a mutated gene for the enzyme phenylalanine hydroxylase (PAH), which converts the amino acid phenylalanine ("Phe ... Phenylalanine hydroxylase (PAH) gene summary, retrieved September 8, 2006 Pietz J, Kreis R, Rupp A, Mayatepek E, Rating D, ... It is due to mutations in the PAH gene which results in low levels of the enzyme phenylalanine hydroxylase. This results in the ...
Phenylalanine hydroxylase Koizumi S; Matsushima Y; Nagatsu T; Iinuma H; Takeuchi T; Umezawa H (September 1984). "3,4- ... 3,4-Dihydroxystyrene (DHS) is a centrally-acting inhibitor of the enzyme phenylalanine hydroxylase (PH). It is likely that DHS ... a novel microbial inhibitor for phenylalanine hydroxylase and other pteridine-dependent monooxygenases". Biochimica et ...
Deficiency in phenylalanine hydroxylase and transaminase activity". Journal of Pediatrics. 68 (3): 351-360. doi:10.1016/s0022- ...
Phenylalanine hydroxylase (PAH) for conversion of L-phenylalanine (PHE) to L-tyrosine (TYR) Tyrosine hydroxylase (TH) for the ... The first enzyme found to use tetrahydrobiopterin is phenylalanine hydroxylase (PAH). Tetrahydrobiopterin is biosynthesized ... One is to convert amino acids such as phenylalanine, tyrosine, and tryptophan to precursors of dopamine and serotonin, major ... Kaufman, S (1958). "A new cofactor required for the enzymatic conversion of phenylalanine to tyrosine". The Journal of ...
Fusetti, F.; Erlandsen, H; Flatmark, T; Stevens, RC (1998). "Structure of Tetrameric Human Phenylalanine Hydroxylase and Its ... 1995). "Expression of recombinant human phenylalanine hydroxylase as fusion protein in Escherichia coli circumvents proteolytic ... Pro mutant human phenylalanine hydroxylase". European Journal of Biochemistry. 268 (4): 997-1005. doi:10.1046/j.1432-1327.2001. ... "Structure/Function Relationships in Human Phenylalanine Hydroxylase. Effect of Terminal Deletions on the Oligomerization, ...
In mammals, tyrosine can be formed from dietary phenylalanine by the enzyme phenylalanine hydroxylase, found in large amounts ... Tyrosine is created from phenylalanine by hydroxylation by the enzyme phenylalanine hydroxylase. Tyrosine is also ingested ... Insufficient amounts of phenylalanine hydroxylase result in phenylketonuria, a metabolic disorder that leads to intellectual ... The enzyme dopamine hydroxylase requires copper as a cofactor (not shown in the diagram) and DOPA decarboxylase requires PLP ( ...
In 1953, G. A. Jervis identified a defect in the hepatic enzyme phenylalanine hydroxylase (PAH) as a cause of PKU. This led to ... Woo SL, Lidsky AS, Güttler F, Chandra T, Robson KJ (1983). "Cloned human phenylalanine hydroxylase gene allows prenatal ... Guthrie R, Susi A (1963). "A simple phenylalanine method for detecting phenylketonuria in large populations of newborn infants ... Jervis GA (1953). "Phenylpyruvic oligophrenia deficiency of phenylalanine-oxidizing system". Proceedings of the Society for ...
"Phenylalanine hydroxylase-stimulating protein/pterin-4 alpha-carbinolamine dehydratase from rat and human liver. Purification, ... "Homomeric and heteromeric interactions between wild-type and mutant phenylalanine hydroxylase subunits: evaluation of two- ... This gene encodes pterin-4 alpha-carbinolamine dehydratase, an enzyme involved in phenylalanine hydroxylation. A deficiency of ...
Tetrahydrobiopterin works with an enzyme called phenylalanine hydroxylase to process a substance called phenylalanine. ... When tetrahydrobiopterin interacts with phenylalanine hydroxylase, tetrahydrobiopterin is altered and must be recycled to a ... 1998). "Expression of human phenylalanine hydroxylase activity in T lymphocytes of classical phenylketonuria children by ... In the absence of usable tetrahydrobiopterin, the body cannot process phenylalanine correctly. As a result, phenylalanine from ...
The conversion of phe to tyr is catalyzed by the enzyme phenylalanine hydroxylase, a monooxygenase. This enzyme catalyzes the ... Tyrosine MS Spectrum Tyrosine metabolism Phenylalanine and tyrosine biosynthesis Phenylalanine, Tyrosine, and tryptophan ... In dopaminergic cells in the brain, tyrosine is converted to L-DOPA by the enzyme tyrosine hydroxylase (TH). TH is the rate- ... The Recommended Dietary Allowance (RDA) for phenylalanine and tyrosine is 33 mg per kilogram of body weight, or 15 mg per pound ...
The aromatic side chain of phenylalanine is hydroxylated by the enzyme phenylalanine hydroxylase to form tyrosine. The ... "Effect of glucagon on phenylalanine metabolism and phenylalanine-degrading enzymes in the rat". Biochemical Journal. 142 (2): ... 4-Hydroxyphenylpyruvic acid (4-HPPA) is an intermediate in the metabolism of the amino acid phenylalanine. ...
... phenylalanine hydroxylase, the enzyme deficient in PKU) & DHPR (di-hydro-folate reductase, also involved in PKU), as well as ... its Mode of Action on Phenylalanine Hydroxylase, and Importance of Genotypes for Pharmacological Therapy of Phenylketonuria". ...
L-tyrosine is also synthesized within the melanocytes from L-phenylalanine by the enzyme phenylalanine hydroxylase (PAH).:1164 ...
Phenylalanine is converted into tyrosine by the enzyme phenylalanine hydroxylase, with molecular oxygen (O2) and ... Tyrosine is converted into L-DOPA by the enzyme tyrosine hydroxylase, with tetrahydrobiopterin, O2, and probably ferrous iron ( ... The metabolic pathway is: Phenylalanine → Tyrosine → L-DOPA → Dopamine → Norepinephrine Thus the direct precursor of ... formerly known as dopamine β-hydroxylase), with O2 and ascorbic acid as cofactors. Norepinephrine itself can further be ...
Inhibition of phenylalanine hydroxylase, a pterin-requiring monooxygenase, by ouudenone and its derivatives, Journal of ... lesions with inhibitors of tyrosine hydroxylase. Linda Mutter in the WRC Toxicology Section used a spot test for tyrosine on ... Laboratory identified triketone inhibition of tyrosine catabolism in rat liver and also found that tyrosine hydroxylase was not ...
... of alleles at the gene locus for the enzyme phenylalanine hydroxylase (PAH). Many of these alleles produce little or no PAH, as ... Phenylalanine Hydroxylase; PAH -612349 Carr, Steven M. "One Gene, One Enzyme". Memorial University of Newfoundland. "On-line ... and the concentration of phenylalanine in the blood [Phe] is about 60 uM. In untreated persons homozygous for one of the PKU ... a result of which the substrate phenylalanine (Phe) and its metabolic byproducts accumulate in the central nervous system and ...
... is a metabolic disorder in which the individual is missing an enzyme called phenylalanine hydroxylase. Absence of this enzyme ... allows the buildup of phenylalanine, which can lead to mental retardation. Genetic engineering - taking a gene from one ...
Blau N, Ghisla S, Heizmann CW (1993). "Phenylalanine hydroxylase-stimulating protein/pterin-4 alpha-carbinolamine dehydratase ...
Tyrosine is semi-essential; it can be synthesized, but only from phenylalanine. A lack of the enzyme phenylalanine hydroxylase ... Phenylalanine → Tyrosine → Thyroxine Phenylalanine, tryptophan, and histidine are essential amino acids for animals. Since they ... In plants, the shikimate pathway first leads to the formation of chorismate, which is the precursor of phenylalanine, tyrosine ... Examples include: Among 20 standard amino acids: Phenylalanine Tryptophan Tyrosine Histidine Others: Thyroxine 5- ...
In these animals, tyrosine is synthesized from phenylalanine via the enzyme phenylalanine hydroxylase, whereas plants have ... As phenylalanine is an essential amino acid, humans (and other animals) have lost the ability to produce it themselves and must ... This is a necessary process for any organism which needs to convert arogenate into phenylalanine or tyrosine, as arogenate is ... It operates in the phenylalanine and tyrosine biosynthesis pathway. The gene which encodes this enzyme has recently been ...
For example, people with an enzyme deficiency caused by mutations in the enzyme phenylalanine hydroxylase cannot metabolize ... One example is phenylketonuria, a genetic disease characterized by a defective phenylalanine hydroxylase enzyme, which is ... Phenylalanine-restricted tyrosine-supplemented diets are a means to nutritionally treat this monogenic disease. In contrast, ... This results in the accumulation of phenylalanine and its breakdown products in the blood and the decrease in tyrosine, which ...
... distal to phenylalanine hydroxylase (PAH) and proximal to tumor rejection antigen (TRA1) on chromosome 12q22-q23". Genomics. 30 ...
In plants, phenylalanine is converted to 4-coumaroyl-CoA in a series of steps known as the general phenylpropanoid pathway ... using phenylalanine ammonia-lyase, cinnamate-4-hydroxylase, and 4-coumaroyl-CoA-ligase.[12] One molecule of 4-coumaroyl-CoA is ... Eriodictyol is then converted into dihydroquercetin with flavanone 3-hydroxylase, which is then converted into quercetin using ... Naringenin is converted into eriodictyol using flavanoid 3′-hydroxylase. ...