Human Metabolome Database Version 3.5

Showing metabocard for Phenylpyruvic acid (HMDB00205)

Record Information
Version 3.5
Creation Date 2005-11-16 08:48:42 -0700
Update Date 2013-02-08 17:08:04 -0700
HMDB ID HMDB00205
Secondary Accession Numbers
  • HMDB01237
Metabolite Identification
Common Name Phenylpyruvic acid
Description Phenylpyruvic 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
Download: MOL | SDF | PDB | SMILES | InChI
Display: 2D Structure | 3D Structure
Synonyms
  1. 2-Oxo-3-phenylpropanoate
  2. 2-Oxo-3-phenylpropanoic acid
  3. 3-Phenyl-2-oxopropanoate
  4. 3-Phenyl-2-oxopropanoic acid
  5. 3-Phenylpyruvate
  6. 3-Phenylpyruvic acid
  7. alpha-Ketohydrocinnamate
  8. alpha-Ketohydrocinnamic acid
  9. b-Phenylpyruvate
  10. b-Phenylpyruvic acid
  11. beta-Phenylpyruvate
  12. beta-Phenylpyruvic acid
  13. Keto-Phenylpyruvate
  14. Phenylpyroracemate
  15. Phenylpyroracemic acid
  16. Phenylpyruvate
Chemical Formula C9H8O3
Average Molecular Weight 164.158
Monoisotopic Molecular Weight 164.047344122
IUPAC Name 2-oxo-3-phenylpropanoic acid
Traditional IUPAC Name 3-phenylpyruvic acid
CAS Registry Number 156-06-9
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 Key BTNMPGBKDVTSJY-UHFFFAOYSA-N
Chemical Taxonomy
Kingdom Organic Compounds
Super Class Aromatic Homomonocyclic Compounds
Class Benzyl Alcohols and Derivatives
Sub Class Phenylpyruvic Acid Derivatives
Other Descriptors
  • Organic Compounds
  • oxo carboxylic acid(ChEBI)
Substituents
  • Alpha Keto Acid
  • Carboxylic Acid
  • Ketone
Direct Parent Phenylpyruvic Acid Derivatives
Ontology
Status Detected 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 Not Available
Cellular locations
  • Cytoplasm
  • Mitochondria
Physical Properties
State Solid
Experimental Properties
Property Value Reference
Melting Point 154 °C Not Available
Boiling Point Not Available Not Available
Water Solubility 112 mg/mL Not Available
LogP Not Available Not Available
Predicted Properties
Property Value Source
Water Solubility 0.93 g/L ALOGPS
LogP 1.30 ALOGPS
LogP 1.9 ChemAxon
LogS -2.25 ALOGPS
pKa (strongest acidic) 3.33 ChemAxon
pKa (strongest basic) -9.8 ChemAxon
Hydrogen Acceptor Count 3 ChemAxon
Hydrogen Donor Count 1 ChemAxon
Polar Surface Area 54.37 A2 ChemAxon
Rotatable Bond Count 3 ChemAxon
Refractivity 42.71 ChemAxon
Polarizability 15.77 ChemAxon
Formal Charge 0 ChemAxon
Physiological Charge -1 ChemAxon
Spectra
1H NMR Spectrum
MS/MS Spectrum Quattro_QQQ 10
MS/MS Spectrum Quattro_QQQ 25
MS/MS Spectrum Quattro_QQQ 40
MS/MS Spectrum GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies)
MS/MS Spectrum GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies )
MS/MS Spectrum GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies )
MS/MS Spectrum GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies )
MS/MS Spectrum GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies)
MS/MS Spectrum GC-MS
MS/MS Spectrum GC-MS
MS/MS Spectrum GC-MS
[1H,13C] 2D NMR Spectrum
Biological Properties
Cellular Locations
  • Cytoplasm
  • Mitochondria
Biofluid Locations
  • Blood
  • Urine
Tissue Location Not Available
Pathways
Name SMPDB Link KEGG Link
Phenylalanine and Tyrosine Metabolism SMP00008 map00360 Link_out
Normal Concentrations
Biofluid Status Value Age Sex Condition Reference
Blood Detected and Quantified
0.5 +/- 0.1 uM Adult (>18 years old) Both Normal
Urine Detected and Quantified
0.17 (0.05-0.67) umol/mmol creatinine Adult (>18 years old) Male Normal
Urine Detected and Quantified
0.24 (0.10-0.76) umol/mmol creatinine Adult (>18 years old) Female Normal
Urine Detected and Quantified
2.00 (0.00-4.00) umol/mmol creatinine Adult (>18 years old) Both Normal
Urine Detected and Quantified
0.25 +/- 0.43 umol/mmol creatinine Infant (0-1 year old) Both Normal
Abnormal Concentrations
Biofluid Status Value Age Sex Condition Reference
Blood Detected and Quantified 50.0 (40.0-60.0) uM Newborn (0-30 days old) Both Phenylketonuria (PKU)
Blood Detected and Quantified 1.4 +/- 0.2 uM Adult (>18 years old) Both Primary biliary cirrhosis
Urine Detected and Quantified 650.00 (300.00-1000.00) umol/mmol creatinine Newborn (0-30 days old) Both Phenylketonuria
Urine Detected and Quantified 146.6 +/- 244.6 umol/mmol creatinine Newborn (0-30 days old) Both Phenylketonuria
Associated Disorders and Diseases
Disease References
Phenylketonuria
  • 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 Link_out
  • 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 Link_out
    Primary biliary cirrhosis
    • 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 Link_out
        Associated OMIM IDs
        DrugBank ID Not Available
        DrugBank Metabolite ID Not Available
        Phenol Explorer Compound ID Not Available
        Phenol Explorer Metabolite ID Not Available
        FoodDB ID FDB020197
        KNApSAcK ID C00000751 Link_out
        Chemspider ID 972 Link_out
        KEGG Compound ID C00166 Link_out
        BioCyc ID PHENYL-PYRUVATE Link_out
        BiGG ID 34111 Link_out
        Wikipedia Link Not Available
        NuGOwiki Link HMDB00205 Link_out
        Metagene Link HMDB00205 Link_out
        METLIN ID 328 Link_out
        PubChem Compound 997 Link_out
        PDB ID PPY Link_out
        ChEBI ID 30851 Link_out
        References
        Synthesis Reference Li, 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. Lasala JM, Coscia CJ: Accumulation of a tetrahydroisoquinoline in phenylketonuria. Science. 1979 Jan 19;203(4377):283-4. Pubmed: 153583 Link_out
        2. 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 Link_out
        3. 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 Link_out
        4. Michals K, Matalon R: Phenylalanine metabolites, attention span and hyperactivity. Am J Clin Nutr. 1985 Aug;42(2):361-5. Pubmed: 4025205 Link_out
        5. 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 Link_out
        6. 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 Link_out
        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 Link_out

        Enzymes
        Name: Macrophage migration inhibitory factor
        Reactions:
        Phenylpyruvic acid unknown Enol-phenylpyruvate details
        Phenylpyruvic acid unknown Enol-phenylpyruvate details
        Gene Name: MIF
        Uniprot ID: P14174 Link_out
        Protein Sequence: FASTA
        Gene Sequence: FASTA
        Name: Tyrosine aminotransferase
        Reactions:
        L-Phenylalanine + Oxoglutaric acid unknown Phenylpyruvic acid + L-Glutamic acid details
        Gene Name: TAT
        Uniprot ID: P17735 Link_out
        Protein Sequence: FASTA
        Gene Sequence: FASTA
        Name: Aspartate aminotransferase, cytoplasmic
        Reactions:
        L-Phenylalanine + Oxoglutaric acid unknown Phenylpyruvic acid + L-Glutamic acid details
        Gene Name: GOT1
        Uniprot ID: P17174 Link_out
        Protein Sequence: FASTA
        Gene Sequence: FASTA
        Name: Aspartate aminotransferase, mitochondrial
        Reactions:
        L-Phenylalanine + Oxoglutaric acid unknown Phenylpyruvic acid + L-Glutamic acid details
        Gene Name: GOT2
        Uniprot ID: P00505 Link_out
        Protein Sequence: FASTA
        Gene Sequence: FASTA
        Name: Kynurenine--oxoglutarate transaminase 1
        Reactions:
        L-Glutamine + Phenylpyruvic acid unknown 2-Keto-glutaramic acid + L-Phenylalanine details
        Gene Name: CCBL1
        Uniprot ID: Q16773 Link_out
        Protein Sequence: FASTA
        Gene Sequence: FASTA
        Name: 4-hydroxyphenylpyruvate dioxygenase
        Reactions:
        Phenylpyruvic acid + Oxygen unknown Ortho-Hydroxyphenylacetic acid + Carbon dioxide details
        Gene Name: HPD
        Uniprot ID: P32754 Link_out
        Protein Sequence: FASTA
        Gene Sequence: FASTA
        Name: L-amino-acid oxidase
        Reactions:
        L-Phenylalanine + Water + Oxygen unknown Phenylpyruvic acid + Ammonia + Hydrogen peroxide details
        Gene Name: IL4I1
        Uniprot ID: Q96RQ9 Link_out
        Protein Sequence: FASTA
        Gene Sequence: FASTA