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Record Information
Version3.6
Creation Date2005-11-16 15:48:42 UTC
Update Date2014-08-06 20:30:15 UTC
HMDB IDHMDB00205
Secondary Accession Numbers
  • HMDB01237
  • HMDB31629
Metabolite Identification
Common NamePhenylpyruvic acid
DescriptionPhenylpyruvic acid is a keto-acid that is an intermediate or catabolic byproduct of phenylalanine metabolism. It has a slight honey-like odor. Levels of phenylpyruvate are normally very low in blood or urine. High levels of phenylpyruvic acid can be found in the urine of individuals with phenylketonuria (PKU). PKU is due to lack of the enzyme phenylalanine hydroxylase (PAH), so that phenylalanine is converted not to tyrosine but to phenylpyruvic acid. In particular, excessive phenylalanine can be metabolized into phenylketones through, a transaminase pathway route involving glutamate. Metabolites of this transamination reaction include phenylacetate, phenylpyruvate and phenethylamine. In persons with PKU, dietary phenylalanine either accumulates in the body or some of it is converted to phenylpyruvic acid. Individuals with PKU tend to excrete large quantities of phenylpyruvate, phenylacetate and phenyllactate, along with phenylalanine, in their urine. If untreated, mental retardation effects and microcephaly are evident by the first year along with other symptoms which include: unusual irritability, epileptic seizures and skin lesions. Hyperactivity, EEG abnormalities and seizures, and severe learning disabilities are major clinical problems later in life. A "musty or mousy" odor of skin, hair, sweat and urine (due to phenylacetate accumulation); and a tendency to hypopigmentation and eczema are also observed. The neural-development effects of PKU are primarily due to the disruption of neurotransmitter synthesis. In particular, phenylalanine is a large, neutral amino acid which moves across the blood-brain barrier (BBB) via the large neutral amino acid transporter (LNAAT). Excessive phenylalanine in the blood saturates the transporter. Thus, excessive levels of phenylalanine significantly decrease the levels of other LNAAs in the brain. But since these amino acids are required for protein and neurotransmitter synthesis, phenylalanine accumulation disrupts brain development, leading to mental retardation.
Structure
Thumb
Synonyms
  1. 2-Hydroxy-3-phenyl-2-propenoic acid, 9CI
  2. 2-Oxo-3-phenylpropanoate
  3. 2-Oxo-3-phenylpropanoic acid
  4. 2-Oxo-3-phenylpropanoic acid (Mixture oxo and keto)
  5. 3-Phenyl-2-oxopropanoate
  6. 3-Phenyl-2-oxopropanoic acid
  7. 3-Phenylpyruvate
  8. 3-Phenylpyruvic acid
  9. a-Hydroxycinnamic acid
  10. a-Oxobenzenepropanoic acid, 9CI
  11. alpha-Ketohydrocinnamate
  12. alpha-Ketohydrocinnamic acid
  13. b-Phenylpyruvate
  14. b-Phenylpyruvic acid
  15. beta-Phenylpyruvate
  16. beta-Phenylpyruvic acid
  17. FEMA 3892
  18. Keto-Phenylpyruvate
  19. Phenylpyroracemate
  20. Phenylpyroracemic acid
  21. Phenylpyruvate
Chemical FormulaC9H8O3
Average Molecular Weight164.158
Monoisotopic Molecular Weight164.047344122
IUPAC Name2-oxo-3-phenylpropanoic acid
Traditional Name3-phenylpyruvic acid
CAS Registry Number156-06-9
SMILES
OC(=O)C(=O)CC1=CC=CC=C1
InChI Identifier
InChI=1S/C9H8O3/c10-8(9(11)12)6-7-4-2-1-3-5-7/h1-5H,6H2,(H,11,12)
InChI KeyBTNMPGBKDVTSJY-UHFFFAOYSA-N
Chemical Taxonomy
KingdomOrganic Compounds
Super ClassAromatic Homomonocyclic Compounds
ClassBenzyl Alcohols and Derivatives
Sub ClassPhenylpyruvic Acid Derivatives
Other Descriptors
  • Organic Compounds
  • oxo carboxylic acid(ChEBI)
Substituents
  • Alpha Keto Acid
  • Carboxylic Acid
  • Ketone
Direct ParentPhenylpyruvic Acid Derivatives
Ontology
StatusDetected and Quantified
Origin
  • Endogenous
  • Microbial
Biofunction
  • Component of Novobiocin biosynthesis
  • Component of Phenylalanine metabolism
  • Component of Phenylalanine, tyrosine and tryptophan biosynthesis
  • Component of Tyrosine metabolism
Application
  • Flavoring Agent
Cellular locations
  • Cytoplasm
  • Mitochondria
Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting Point154 °CNot Available
Boiling PointNot AvailableNot Available
Water Solubility112 mg/mLNot Available
LogPNot AvailableNot Available
Predicted Properties
PropertyValueSource
Water Solubility0.93 g/LALOGPS
logP1.3ALOGPS
logP1.9ChemAxon
logS-2.2ALOGPS
pKa (Strongest Acidic)3.33ChemAxon
pKa (Strongest Basic)-9.8ChemAxon
Physiological Charge-1ChemAxon
Hydrogen Acceptor Count3ChemAxon
Hydrogen Donor Count1ChemAxon
Polar Surface Area54.37ChemAxon
Rotatable Bond Count3ChemAxon
Refractivity42.71ChemAxon
Polarizability15.77ChemAxon
Spectra
SpectraGC-MSMS/MS1D NMR2D NMR
Biological Properties
Cellular Locations
  • Cytoplasm
  • Mitochondria
Biofluid Locations
  • Blood
  • Urine
Tissue LocationNot Available
Pathways
NameSMPDB LinkKEGG Link
Phenylalanine and Tyrosine MetabolismSMP00008map00360
Normal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified0.5 +/- 0.1 uMAdult (>18 years old)BothNormal details
UrineDetected and Quantified0.17 (0.05-0.67) umol/mmol creatinineAdult (>18 years old)MaleNormal details
UrineDetected and Quantified0.24 (0.10-0.76) umol/mmol creatinineAdult (>18 years old)FemaleNormal details
UrineDetected and Quantified2.00 (0.00-4.00) umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified0.25 +/- 0.43 umol/mmol creatinineInfant (0-1 year old)BothNormal details
Abnormal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified50.0 (40.0-60.0) uMNewborn (0-30 days old)BothPhenylketonuria details
BloodDetected and Quantified1.4 +/- 0.2 uMAdult (>18 years old)BothPrimary biliary cirrhosis details
UrineDetected and Quantified650.00 (300.00-1000.00) umol/mmol creatinineNewborn (0-30 days old)BothPhenylketonuria details
UrineDetected and Quantified146.6 +/- 244.6 umol/mmol creatinineNewborn (0-30 days old)BothPhenylketonuria details
Associated Disorders and Diseases
Disease References
Phenylketonuria
  1. Langenbeck U, Behbehani A, Luthe H: Renal transport of aromatic acids in patients with phenylketonuria. J Inherit Metab Dis. 1981;4(2):69-70. Pubmed: 6790852
  2. Monch E, Kneer J, Jakobs C, Arnold M, Diehl H, Batzler U: Examination of urine metabolites in the newborn period and during protein loading tests at 6 months of age--Part 1. Eur J Pediatr. 1990;149 Suppl 1:S17-24. Pubmed: 2091926
  3. MetaGene
Primary biliary cirrhosis
  1. Turchany JM, Leung PS, Iwayama T, Jefferson DM, Ishida J, Yamaguchi M, Munoz S, Danner DJ, Dickson ER, Gershwin ME: Comparative metabolism and structure of BCKD-E2 in primary biliary cirrhosis. J Autoimmun. 1993 Aug;6(4):459-66. Pubmed: 8216688
Associated OMIM IDs
DrugBank IDNot Available
DrugBank Metabolite IDNot Available
Phenol Explorer Compound IDNot Available
Phenol Explorer Metabolite IDNot Available
FoodDB IDFDB020197
KNApSAcK IDC00000751
Chemspider ID972
KEGG Compound IDC00166
BioCyc IDPHENYL-PYRUVATE
BiGG ID34111
Wikipedia LinkNot Available
NuGOwiki LinkHMDB00205
Metagene LinkHMDB00205
METLIN ID328
PubChem Compound997
PDB IDPPY
ChEBI ID30851
References
Synthesis ReferenceLi, Hongbin; Luo, Yuzhong. Preparation of phenyl-pyruvic acid by dicarbonylation of benzyl halide. Faming Zhuanli Shenqing Gongkai Shuomingshu (1996), 5 pp.
Material Safety Data Sheet (MSDS)Download (PDF)
General References
  1. Shoemaker JD, Elliott WH: Automated screening of urine samples for carbohydrates, organic and amino acids after treatment with urease. J Chromatogr. 1991 Jan 2;562(1-2):125-38. Pubmed: 2026685
  2. Lee SH, Kim SO, Chung BC: Gas chromatographic-mass spectrometric determination of urinary oxoacids using O-(2,3,4,5,6-pentafluorobenzyl)oxime-trimethylsilyl ester derivatization and cation-exchange chromatography. J Chromatogr B Biomed Sci Appl. 1998 Nov 20;719(1-2):1-7. Pubmed: 9869358
  3. Boulat O, Gradwohl M, Matos V, Guignard JP, Bachmann C: Organic acids in the second morning urine in a healthy Swiss paediatric population. Clin Chem Lab Med. 2003 Dec;41(12):1642-58. Pubmed: 14708889
  4. Lasala JM, Coscia CJ: Accumulation of a tetrahydroisoquinoline in phenylketonuria. Science. 1979 Jan 19;203(4377):283-4. Pubmed: 153583
  5. Cassidei L, Dell'atti A, Sciacovelli O: Improvement of the FeCl3 test for phenylpyruvic acid. Clin Chim Acta. 1978 Dec 1;90(2):121-7. Pubmed: 719897
  6. Michals K, Matalon R: Phenylalanine metabolites, attention span and hyperactivity. Am J Clin Nutr. 1985 Aug;42(2):361-5. Pubmed: 4025205
  7. Nakahara T, Ishida J, Yamaguchi M, Nakamura M: Determination of alpha-keto acids including phenylpyruvic acid in human plasma by high-performance liquid chromatography with chemiluminescence detection. Anal Biochem. 1990 Nov 1;190(2):309-13. Pubmed: 2291475
  8. Yannai, Shmuel. (2004) Dictionary of food compounds with CD-ROM: Additives, flavors, and ingredients. Boca Raton: Chapman & Hall/CRC.

Enzymes

General function:
Involved in cell surface binding
Specific function:
Pro-inflammatory cytokine. Involved in the innate immune response to bacterial pathogens. The expression of MIF at sites of inflammation suggests a role as mediator in regulating the function of macrophages in host defense. Counteracts the anti-inflammatory activity of glucocorticoids. Has phenylpyruvate tautomerase and dopachrome tautomerase activity (in vitro), but the physiological substrate is not known. It is not clear whether the tautomerase activity has any physiological relevance, and whether it is important for cytokine activity.
Gene Name:
MIF
Uniprot ID:
P14174
Molecular weight:
12476.19
Reactions
Phenylpyruvic acid → Enol-phenylpyruvatedetails
Phenylpyruvic acid → Enol-phenylpyruvatedetails
General function:
Involved in 1-aminocyclopropane-1-carboxylate synthase activity
Specific function:
Transaminase involved in tyrosine breakdown. Converts tyrosine to p-hydroxyphenylpyruvate. Can catalyze the reverse reaction, using glutamic acid, with 2-oxoglutarate as cosubstrate (in vitro). Has much lower affinity and transaminase activity towards phenylalanine.
Gene Name:
TAT
Uniprot ID:
P17735
Molecular weight:
50398.895
Reactions
L-Phenylalanine + Oxoglutaric acid → Phenylpyruvic acid + L-Glutamic aciddetails
General function:
Involved in transferase activity, transferring nitrogenous groups
Specific function:
Plays a key role in amino acid metabolism (By similarity).
Gene Name:
GOT1
Uniprot ID:
P17174
Molecular weight:
46247.14
Reactions
L-Phenylalanine + Oxoglutaric acid → Phenylpyruvic acid + L-Glutamic aciddetails
General function:
Involved in transferase activity, transferring nitrogenous groups
Specific function:
Catalyzes the irreversible transamination of the L-tryptophan metabolite L-kynurenine to form kynurenic acid (KA). Plays a key role in amino acid metabolism. Important for metabolite exchange between mitochondria and cytosol. Facilitates cellular uptake of long-chain free fatty acids.
Gene Name:
GOT2
Uniprot ID:
P00505
Molecular weight:
47517.285
Reactions
L-Phenylalanine + Oxoglutaric acid → Phenylpyruvic acid + L-Glutamic aciddetails
General function:
Involved in 1-aminocyclopropane-1-carboxylate synthase activity
Specific function:
Catalyzes the irreversible transamination of the L-tryptophan metabolite L-kynurenine to form kynurenic acid (KA). Metabolizes the cysteine conjugates of certain halogenated alkenes and alkanes to form reactive metabolites. Catalyzes the beta-elimination of S-conjugates and Se-conjugates of L-(seleno)cysteine, resulting in the cleavage of the C-S or C-Se bond.
Gene Name:
CCBL1
Uniprot ID:
Q16773
Molecular weight:
47874.765
Reactions
L-Glutamine + Phenylpyruvic acid → 2-Keto-glutaramic acid + L-Phenylalaninedetails
General function:
Involved in 4-hydroxyphenylpyruvate dioxygenase activity
Specific function:
Key enzyme in the degradation of tyrosine.
Gene Name:
HPD
Uniprot ID:
P32754
Molecular weight:
40497.105
Reactions
Phenylpyruvic acid + Oxygen → Ortho-Hydroxyphenylacetic acid + Carbon dioxidedetails
General function:
Involved in oxidoreductase activity
Specific function:
Lysosomal L-amino-acid oxidase with highest specific activity with phenylalanine. May play a role in lysosomal antigen processing and presentation (By similarity).
Gene Name:
IL4I1
Uniprot ID:
Q96RQ9
Molecular weight:
65327.26
Reactions
L-Phenylalanine + Water + Oxygen → Phenylpyruvic acid + Ammonia + Hydrogen peroxidedetails