Phenylpyruvic Acids
Involvement of manganese in conversion of phenylalanine to benzaldehyde by lactic acid bacteria. (1/75)
We examined the involvement of Mn(II) in the conversion of phenylalanine to benzaldehyde in cell extracts of lactic acid bacteria. Experiments performed with Lactobacillus plantarum demonstrated that Mn(II), present at high levels in this strain, is involved in benzaldehyde formation by catalyzing the conversion of phenylpyruvic acid. Experiments performed with various lactic acid bacterial strains belonging to different genera revealed that benzaldehyde formation in a strain was related to a high Mn(II) level. (+info)Inhibition of transketolase by p-hydroxyphenylpyruvate. (2/75)
The effect of p-hydroxyphenylpyruvate, a natural analogue of transketolase substrate, on the catalytic activity of the enzyme was investigated. p-Hydroxyphenylpyruvate proved to be a reversible and competitive inhibitor of transketolase with respect to substrate; it was also able to displace thiamine diphosphate from holotransketolase. The data suggest that p-hydroxyphenylpyruvate participates in the regulation of tyrosine biosynthesis by influencing the catalytic activity of transketolase. (+info)Metabolism of carnitine in phenylacetic acid-treated rats and in patients with phenylketonuria. (3/75)
The effect of metabolites accumulating in phenylketonuria (PKU) was investigated on carnitine metabolism in rats and in patients with PKU. Of phenylacetic acid (PEAA), phenylpyruvic acid and homogentisic acid the PEAA was found to be the most effective in inhibiting carnitine biosynthesis in rats. Following 60 min, a single intraperitoneal dose of PEAA the relative conversion rate, i. e. the hydroxylation, of tracer [Me-(3)H]butyrobetaine to [Me-(3)H]carnitine decreased from 62.2+/-6.00% to 39.4+/-5.11% (means+/-S.E.M., P<0.01) in the liver, in the only organ doing this conversion in rats. The conversion of loading amount of unlabeled butyrobetaine to carnitine was also markedly reduced. The impaired hydroxylation of butyrobetaine was reflected by a reduced free and total carnitine levels in the liver and a reduced total carnitine concentration in the plasma. PEAA decreased the hepatic level of glutamic acid and alpha-ketoglutaric acid (alpha-KG), suggesting a mechanism for the reduced flux through the butyrobetaine hydroxylase enzyme, because alpha-KG is an obligatory co-enzyme. In the plasma and urine of PKU patients on unrestricted diet, markedly decreased total carnitine levels were detected. In the liver of PEAA-treated rats and urine of PKU patients, a novel carnitine derivative, phenacetyl-carnitine was verified by HPLC and gas chromatography-mass spectrometry. (+info)Gas-liquid chromatography of phenylalanine and its metabolites in serum and urine of various hyperphenylalaninemic subjects, their relatives, and controls. (4/75)
Phenylalanine and its metabolites were determined in serum and urine of phenylketonuric subjects and in subjects with milder hyperphenylalaninemia in whom blood phenylalanine concentrations were usually less than 200 mg/liter. Metabolite concentrations were related to serum phenylalanine, and in hyperphenylalaninemic subjects were between those for treated and untreated phenylketonuric subjects. Phenyllactic and phenylpyruvic acids were excreted by all of the mild hyperphenylalaninemic subjects except for the youngest (one-year-old twins) and the only subject with a serum phenylalanine of less than 100 mg/liter. Serum and urinary metabolites of heterozygotes of both conditions were similar before and after a phenylalanine load. The similar pattern of metabolites in phenylketonuric and mild hyperphenylalaninemic subjects reinforces the belief that the latter have some phenylalanine hydroxylase activity, and that this is the essential difference between the two groups. (+info)The mitochondrial pyruvate carrier. Kinetics and specificity for substrates and inhibitors. (5/75)
1. Studies on the kinetics of pyruvate transport into mitochondria by an 'inhibitor-stop' technique were hampered by the decarboxylation of pyruvate by mitochondria even in the presence of rotenone. Decarboxylation was minimal at 6 degrees C. At this temperature the Km for pyruvate was 0.15 mM and Vmax. was 0.54nmol/min per mg of protein; alpha-cyano-4-hydroxycinnamate was found to be a non-competitive inhibitor, Ki 6.3 muM, and phenyl-pyruvate a competitive inhibitor, Ki 1.8 mM. 2. At 100 muM concentration, alpha-cyano-4-hydroxycinnamate rapidly and almost totally inhibited O2 uptake by rat heart mitochondria oxidizing pyruvate. Inhibition could be detected at concentrations of inhibitor as low as 1 muM although inhibition took time to develop at this concentration. Inhibition could be reversed by diluting out the inhibitor. 3. Various analogues of alpha-cyano-4-hydroxycinnamate were tested on rat liver and heart mitochondria. The important structural features appeared to be the alpha-cyanopropenoate group and the hydrophobic aromatic side chain. Alpha-Cyanocinnamate, alpha-cyano-5-phenyl-2,4-pentadienoate and compound UK 5099 [alpha-cyano-beta-(2-phenylindol-3-yl)acrylate] were all more powerful inhibitors than alpha-cyano-4-hydroxycinnamate showing 50% inhibition of pyruvate-dependent O2 consumption by rat heart mitochondria at concentrations of 200, 200 and 50 nM respectively. 4. The specificity of the carrier for its substrate was studied by both influx and efflux experiments. Oxamate, 2-oxobutyrate, phenylpyruvate, 2-oxo-4-methyl-pentanoate, chloroacetate, dichloroacetate, difluoroacetate, 2-chloropropionate, 3-chloropropionate and 2,2-dichloropropionate all exchanged with pyruvate, whereas acetate, lactate and trichloroacetate did not. 5. Pyruvate entry into the mitochondria was shown to be accompanied by the transport of a proton (or by exchange with an OH-ion). This proton flux was inhibited by alpha-cyano-4-hydroxycinnamate and allowed measurements of pyruvate transport at higher temperatures to be made. The activation energy of mitochondrial pyruvate transport was found to be 113 kJ (27 kcal)/mol and by extrapolation the rate of transport of pyruvate at 37 degrees C to be 42 nmol/min per mg of protein. The possibility that pyruvate transport into mitochondria may be rate limiting and involved in the regulation of gluconegenesis is discussed. 6. The transport of various monocarboxylic acids into mitochondria was studied by monitoring proton influx. The transport of dichloroacetate, difluoroacetate and oxamate appeared to be largely dependent on the pyruvate carrier and could be inhibited by pyruvate-transport inhibitors. However, many other halogenated and 2-oxo acids which could exchange with pyruvate on the carrier entered freely even in the presence of inhibitor. (+info)Phenylalanine hydroxylase from Pseudomonas sp. (ATCC 11299a). Purification, molecular weight, and influence of tyrosine metabolites on activation and hydroxylation. (6/75)
Phenylalanine hydroxylase from Pseudomonas sp. (ATCC 11299a) has been purified 25- to 30-fold by a procedure which has been modified from that previously described for this organism (Guroff, G., and Ito, T. (1965) J. Biol. Chem. 240, 1175-1184; Guroff, G., and Rhoads, C. A. (1967) J. Biol. Chem. 242, 3641-3645). Further purification yielded a preparation which was judged to be about 80% pure by sodium dodecyl sulfate-containing and standard analytical polyacrylamide gels, but the activity in this preparation has proved to be very labile. The enzyme appears to be a single protein chain of between 25,000 to 27,000 molecular weight. Phenylalanine, tyrosine, and tryptophan inhibit the activation of the enzyme by iron in a competitive fashion. The tyrosine metabolites, p-hydroxyphenylpyruvic and homogentisic acids exhibit a biphasic effect on activation, stimulating at low iron, and inhibiting at higher iron concentrations. The hydroxylation itself is inhibited by tyrosine and related compounds such as L-3,4-dihydroxyphenylalanine and dopamine. p-Hydroxyphenylpyruvic acid is a competitive inhibitor with respect to both substrate and cofactor. The data indicate a variety of means by which the bacterium can regulate phenylalanine hydroxylation. (+info)The Sulphation of p-hydroxyphenylpyruvic acid and related compounds by the rat liver cytosol. (7/75)
Cytosol preparations of rat liver and kidney were examined for their ability to transfer sulphate from adenosine 3'-phosphate 5'-sulphatophosphate to p-hydroxyphenylpyruvic acid. Little activity towards this substrate was observed, and the main product detected in the reaction mixtures was identified as p-hydroxybenzyl alcohol O-sulphate. This was not formed from p-hydroxybenzaldehyde, a spontaneous oxidation product of p-hydroxyphenylpyruvic acid, by sulphation followed by a rapid enzyme-catalysed reduction of the intermediate phydroxybenzaldehyde O-sulphate. This product was formed mainly by this sequence of reactions, but the reverse, reduction followed by sulphation, also appeared possible. p-Hydroxybenzyl alcohol itself was very readily sulphated by both preparations, and the liver also produced a sulpho-conjugate of homogentisic acid. These observations are important in calculating the turnover of L-tyrosine O-sulphate in the mammalian system, and establish that p-hydroxyphenylpyruvic acid O-sulphate is an end product of its metabolism, rather than an intermediate in its synthesis by reversed transamination. (+info)Studies on the experimental phenylketonuria in rats. (8/75)
Wister albino pregnant rats were fed on pellets containing 3.5% L-phenylalanine (Phe) from 10 days before the expected date of birth. The diet was then switched to 7% Phe pellets at the third week after birth. Baby rats were reared with breast milk, and weaned at the end of the 4th week after birth; thereafter, they were reared with a normal diet for one week at the 5th week, and then were given 7% Phe diet from the 6th week. These rats, which were reared with a diet of high Phe, showed a similar metabolic pattern to that of human phenylketonuria (PKU) in the following aspects: definite suppression of the liver Phe hydroxylase activity, excretion of a large amount of phenylpyruvic acid (PPA) and phenyllactic acid (PLA) into urine, and an elvated level of blood Phe content. But, they had an excessive amount of blood tyrosine (Tyr), and concurrently excreted massive homogentisic acid (HGA) in urine just as in human tyrosinemia alkaptonuria. The absence of urinary o-hydroxyphenylacetic acid (o-HPAA) was also a distinct difference from human PKU. In some rats, mild inhibition of the liver Phe hydroxylase activity was observed. In other rats, there was no excretion of PPA into urine as in human hyperphenylalaninemia. Further, the regulatory mechanism of Phe catabolism of experimental PKU was discussed by analysing the enzyme activity of the liver Phe hydroxylase, phenylalanine-pyruvate (Phe-Pyr) transaminase and tyrosine alpha-ketoglutarate (Tyr-alpha-Kg) transaminase at different developmental stages of the rats. (+info)Phenylpyruvic acid is not a medical condition, but rather a chemical compound that is produced in the body. It is a byproduct of phenylalanine metabolism, an essential amino acid that cannot be synthesized by the human body and must be obtained through dietary sources such as proteins.
In some rare genetic disorders, such as phenylketonuria (PKU), the body is unable to properly metabolize phenylalanine due to a deficiency or malfunction of the enzyme phenylalanine hydroxylase. As a result, phenylpyruvic acid and other toxic byproducts accumulate in the body, leading to various health problems such as intellectual disability, seizures, and behavioral issues.
Therefore, the medical relevance of phenylpyruvic acid lies in its association with certain metabolic disorders, particularly PKU, and its potential use as a diagnostic marker for these conditions.
Phenylalanine is an essential amino acid, meaning it cannot be produced by the human body and must be obtained through diet or supplementation. It's one of the building blocks of proteins and is necessary for the production of various molecules in the body, such as neurotransmitters (chemical messengers in the brain).
Phenylalanine has two forms: L-phenylalanine and D-phenylalanine. L-phenylalanine is the form found in proteins and is used by the body for protein synthesis, while D-phenylalanine has limited use in humans and is not involved in protein synthesis.
Individuals with a rare genetic disorder called phenylketonuria (PKU) must follow a low-phenylalanine diet or take special medical foods because they are unable to metabolize phenylalanine properly, leading to its buildup in the body and potential neurological damage.
Phenylpyruvic acid
Ketone
Prephenate dehydratase
Mandelic acid
Phenylpyruvate tautomerase
Terphenylquinones
Nyctinasty
Hippuric acid
Diagnostic microbiology
Ivar Asbjørn Følling
Phenylketonuria
Giuseppe Cilento
Keto acid
Naturally occurring phenols
Robert Guthrie
1,2-Dioxetane
Enterobacteriaceae
C9H8O3
Atromentin
List of MeSH codes (D02)
Phenylpyruvic acid - Wikipedia
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Phenylketonuria3
- When the activity of the enzyme phenylalanine hydroxylase is reduced, the amino acid phenylalanine accumulates and gets converted into phenylpyruvic acid (phenylpyruvate), which leads to 'Phenylketonuria (PKU)' instead of 'tyrosine' which is the normal product of phenylalanine hydroxylase. (wikipedia.org)
- High values of mandelic acid also occur in phenylketonuria (PKU). (healthmatters.io)
- Phenylketonuria ( PKU ) is an inborn error of metabolism that results in decreased metabolism of the amino acid phenylalanine . (mdwiki.org)
Derivatives5
- Classically it is produced from aminocinnamic acid derivatives. (wikipedia.org)
- It has been prepared by condensation of benzaldehyde and glycine derivatives to give phenylazlactone, which is then hydrolyzed with acid- or base-catalysis. (wikipedia.org)
- Oliver, Douglas W. "Phenylpyruvic acid derivatives as enzyme inhibitors: Therapeutic potential on macrophage migration inhibitory factor" Medicinal Chemistry Research 2004, volume 13, pp. 565-577. (wikipedia.org)
- A group of compounds that are derivatives of phenylpyruvic acid which has the general formula C6H5CH2COCOOH, and is a metabolite of phenylalanine. (nih.gov)
- Derivatives of PYRUVIC ACID , including its salts and esters. (nih.gov)
Oxidase6
- The method uti- lizes L-amino acid oxidase from snake venom to oxidize L-phenylalanine to phenylpyruvic acid. (nih.gov)
- Catalase is added to protect the a-keto acid from peroxide formed in the oxidative deamination by L-amino acid oxidase. (nih.gov)
- Enol-borate complexes have been employed by Knox and Pitt® to determine p-hydroxyphenylpyruvie acid oxidase activity in mammalian liver preparations. (nih.gov)
- sodium arsenate [Na,HAsO,-7H.0]) was adjusted to pH 6.5 with HCl and made up to 1 L. 4, Snake venom L-amino acid oxidase (the venom of Crotalus adamanteus*): a suspen- sion of dried venom in water was made which contained 10 mg. per milliliter. (nih.gov)
- The activity of the L-amino acid oxidase preparation can be assayed by using 0.1 ml. of the standard phenylalanine solution in place of serum in the directions given below. (nih.gov)
- In a poorly understood complex reaction, the enzyme phenylpyruvic acid oxidase is thought simultaneously to move the pyruvic acid side chain, to decarboxylate it, and to add an additional hydroxyl group to the ring. (medscape.com)
Phenyllactic2
- Exposure to Phe or to six Phe metabolites [phenylacetic acid (PAA), phenyllactic acid, hydroxyphenylacetic acid, phenylpyruvic acid, phenylethylamine (PEA), and mandelic acid] did not result in astroglial or neuronal cell cytotoxicity. (nih.gov)
- Normal phenyllactic and phenylpyruvic acids indicate that styrene or drug exposure is more likely than PKU as a cause of these abnormalities. (healthmatters.io)
Homogentisic acid4
- The normal catabolic pathway, which moves tyrosine to 4-hydroxyphenylpyruvic acid, then to homogentisic acid, the ring then being cleaved to produce maleylacetoacetate (MAA) and fumarylacetoacetate (FAA), is interrupted at the next step, which would normally produce fumarate and acetoacetate. (medscape.com)
- The product, homogentisic acid, is actually ortho-meta- dihydroxyphenylacetic acid. (medscape.com)
- A deficiency of the hepatic enzyme homogentisate 1,2-dioxygenase (HGO) forces the accumulation of homogentisic acid, which is rapidly cleared in the kidney and excreted. (medscape.com)
- Upon contact with air, homogentisic acid is oxidized to form a pigmentlike polymeric material responsible for the black color of standing urine. (medscape.com)
Mandelic acid3
- Mandelic acid is the major metabolite of styrene. (healthmatters.io)
- When exposed to 100 ppm of styrene in air, mandelic acid in urine was found to average 1700 mmol/mol creatinine. (healthmatters.io)
- Mandelic acid is also a metabolite of ethylbenzene, and some antispasmodic and vasodilator drugs. (healthmatters.io)
Amino acid phenylalanine2
- PAG is a byproduct of the breakdown of the essential amino acid phenylalanine by gut microbes. (medscape.com)
- The phenylalanine hydroxylase (PAH) enzyme performs the breakdown of the amino acid phenylalanine (phe) into tyrosine (Tyr), which is required by the body to produce stress neurotransmitters such as epinephrine, norepinephrine and dopamine. (elioacademy.org)
Tyrosine1
- More recently these complexes have been used to measure the activity of several enzymes whieh either form or break down the aromatic a-keto acids related to phenylalanine, tyrosine, tryptophan, and histidine. (nih.gov)
Urine2
- The disease may present clinically with seizures , hypopigmentation (excessively fair hair and skin), and a "musty odor" to the baby's sweat and urine (due to phenylacetate , a carboxylic acid produced by the oxidation of phenylketone). (mdwiki.org)
- However, if left undiagnosed, patients will have high concentrations of phenylalanine in their blood and will excrete phenylpyruvic acid, leading to discolored urine. (elioacademy.org)
Compounds1
- The combinational actions of ethylene and MeJA effectively promoted the total phenolic compounds, especially the C6C1 compounds (such as salicylic acid, benzoic acid) and C6C3 ones (such as cinnamic acid, sinapic acid). (frontiersin.org)
Phenylacetic acid2
- Unabsorbed phenylalanine that reaches the large intestine can be metabolized by gut microbiota to form phenylpyruvic acid (the initial microbiota-generated deamination product) and subsequently phenylacetic acid. (medscape.com)
- After going through the liver, phenylacetic acid is metabolized to produce PAG. (medscape.com)
Pyruvic acid derivative1
- Hydrogen tunneling avoided: enol-formation from a charge-tagged phenyl pyruvic acid derivative evidenced by tandem-MS, IR ion spectroscopy and theory. (uni-koeln.de)
Alcohols2
- Sterols, triterpenic alcohols and triterpenic acids remained unchanged, and only slight losses of squalene (8 wt%) and α-tocopherol (13 wt%) were observed in OPO after processing and storage, respectively. (bvsalud.org)
- A total of 66 volatile components were detected in Zhenghe white peony tea with different storage years, mainly alcohols, esters and acids, with the highest alcohol content in BMD0 and BMD5, and the highest ester content in BMD10 and BMD15. (tea-science.com)
Enzyme2
- It converts phenylalanine to ammonia and trans-cinnamic acid, so that phenylalanine levels are reduced to normal ranges regardless of the activity of the PAH enzyme or the BH4 cofactor. (elioacademy.org)
- Another common reasons for this elevated ratio is inhibition of this enzyme by Clostridia byproducts including HPHPA , 4-cresol , or 4-hydroxyphenylacetic acid . (healthmatters.io)
Citric acid1
- citric acid 33. (tendersinkarnataka.com)
Fumaric acid1
- Investigators inferred that the enzymatic defect might reside in deficiency of fumarylacetoacetase, which mediates production of fumaric acid and acetoacetate in both liver and kidney. (medscape.com)
Benzoic acid1
- benzoic acid 25. (tendersinkarnataka.com)
Hydrochloric acid2
- hydrochloric acid 5 litre pack 123 ortho phosporic acid 500 ml pack 124 carbolic acid / phenol 500 gm. (uttarpradeshtenders.net)
- hydrochloric acid 5 litre pack , ortho phosporic acid 500 ml pack , carbolic acid / phenol 500 gm. (uttarpradeshtenders.net)
Deficiency1
- Ascorbic acid deficiency may also be related to this abnormality since ascorbic acid is a cofactor for phenylalanine hydroxylase. (healthmatters.io)
Metabolites1
- The Organic Acids Test measures levels of HVA (homovanillic acid) and VMA (vanillylmandelic acid), the metabolites of the neurotransmitters, dopamine and epinephrine/norepinephrine. (healthmatters.io)
Dietary2
Ammonia1
- A pair of principal enzymes, glutamate dehydrogenase and glutamine synthatase, are found in all organisms and effect the conversion of ammonia into the amino acids glutamate and glutamine, respectively. (tdmuv.com)
Protein1
- According to the Hazen, after protein is digested and broken down into individual amino acids, the majority of phenylalanine is absorbed by the small intestine. (medscape.com)
Pathway2
- The therapeutic agent 2-(2-nitro-4-trifluoromethylbenzoyl)-1,3-cyclohexanedione (NTBC) interrupts the pathway after formation of 4-OH-phenylpyruvic acid. (medscape.com)
- e.g., amino acids and vitamins ✓ Assuming that many genes interact in a pathway for synthesis of amino acids and other cellular products, Beadle and Tatum then dissected the biochemical pathways. (slideshare.net)
Organisms1
- Nitrogen fixation is carried out by bacterial nitrogenases forming reduced nitrogen, NH4+ which can then be used by all organisms to form amino acids. (tdmuv.com)
Supplementation2
- Supplementation with ascorbic acid (vitamin C) at 1000 mg/day or more may be beneficial. (healthmatters.io)
- Other treatment options include amino acid supplementation and therapies involving sapropterin and pegvaliase. (elioacademy.org)
Cellular1
- There is a subsequent imbalance in the distribution of many amino acids across cellular membranes and across the blood-brain barrier. (nih.gov)
Supplements1
- For adults with PKU who cannot tolerate a restricted diet, amino acid supplements can be administered. (elioacademy.org)
Aspartame1
- Other causes of an increased ratio include inhibition of DBH by the mold metabolite fusaric acid, pharmaceuticals such as disulfiram, or food additives like aspartame. (healthmatters.io)
Rapidly1
- In the presence of arsenate and borate ions the resulting a-keto acid is rapidly converted to an enol-borate complex which has a high ab- sorption in ultraviolet light. (nih.gov)
Group1
- A 2-oxo monocarboxylic acid that is 3-phenylpropanoic acid substituted by an oxo group at position 2. (chemspider.com)
Description1
- Short description: Arom amin-acid metab NEC. (icd9data.com)