创建日期
2005-11-16 15:48:42 UTC
更新日期
2020-11-09 23:12:43 UTC
二级访问
HMDB0001237
HMDB00205
HMDB0031629
HMDB01237
HMDB31629
描述
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), an inborn error of metabolism. 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. Phenylpyruvic acid is also a microbial metabolite, it can be produced by Lactobacillus plantarum (PMID: 9687465).
同义词
1:3-Phenyl-2-oxopropanoate
2:3-Phenyl-2-oxopropanoic acid
3:3-Phenylpyruvic acid
4:alpha-Ketohydrocinnamic acid
5:alpha-Oxo-benzenepropanoic acid
6:beta-Phenylpyruvic acid
7:Keto-phenylpyruvate
8:Phenylbrenztraubensaeure
9:Phenylpyruvate
10:3-PHENYLPYRUVate
11:a-Ketohydrocinnamate
12:a-Ketohydrocinnamic acid
13:alpha-Ketohydrocinnamate
14:α-Ketohydrocinnamate
15:α-Ketohydrocinnamic acid
16:a-Oxo-benzenepropanoate
17:a-Oxo-benzenepropanoic acid
18:alpha-Oxo-benzenepropanoate
19:α-Oxo-benzenepropanoate
20:α-Oxo-benzenepropanoic acid
21:b-Phenylpyruvate
22:b-Phenylpyruvic acid
23:beta-Phenylpyruvate
24:β-Phenylpyruvate
25:β-Phenylpyruvic acid
26:keto-Phenylpyruvic acid
27:2-Oxo-3-phenylpropanoate
28:2-Oxo-3-phenylpropanoic acid
29:a-Hydroxycinnamic acid
30:FEMA 3892
31:Phenylpyruvic acid
32:2-Oxo-3-phenylpropionic acid
33:3-Phenyl-2-oxopropionic acid
34:alpha-Oxobenzenepropanoic acid
35:alpha-Oxobenzenepropionic acid
36:α-Oxobenzenepropanoic acid
37:α-Oxobenzenepropionic acid
化学名称
2-oxo-3-phenylpropanoic acid
代谢物结构字符串
OC(=O)C(=O)CC1=CC=CC=C1
inchi标识符
InChI=1S/C9H8O3/c10-8(9(11)12)6-7-4-2-1-3-5-7/h1-5H,6H2,(H,11,12)
印记键
BTNMPGBKDVTSJY-UHFFFAOYSA-N
分类学
description: belongs to the class of organic compounds known as phenylpyruvic acid derivatives. Phenylpyruvic acid derivatives are compounds containing a phenylpyruvic acid moiety, which consists of a phenyl group substituted at the second position by an pyruvic acid.
description:Phenylpyruvic acid derivatives
kingdom:Organic compounds
super_class:Benzenoids
class:Benzene and substituted derivatives
sub_class:Phenylpyruvic acid derivatives
molecular_framework:Aromatic homomonocyclic compounds
1:Alpha-hydroxy ketones
2:Alpha-keto acids and derivatives
3:Carboxylic acids
4:Hydrocarbon derivatives
5:Monocarboxylic acids and derivatives
6:Organic oxides
7:Phenylpropanoic acids
8:3-phenylpropanoic-acid
9:Alpha-hydroxy ketone
10:Alpha-keto acid
11:Aromatic homomonocyclic compound
12:Carbonyl group
13:Carboxylic acid
14:Carboxylic acid derivative
15:Hydrocarbon derivative
16:Keto acid
17:Ketone
18:Monocarboxylic acid or derivatives
19:Organic oxide
20:Organic oxygen compound
21:Organooxygen compound
22:Phenylpyruvate
23:2-oxo monocarboxylic acid
本体论
term:Physiological effect
definition:The effect on an organism physiology, resulting from its exposure to a chemical.
parent_id:
level:1
type:parent
term:Health effect
definition:A health condition or observation associated with a stimuli or with a biological activity of a chemical.
parent_id:7693
*level:2
type:parent
term:Health condition
definition:A health effect that consists on short or long-term disease, condition, disorder, syndrome or constant abnormality.
parent_id:7694
**level:3
type:parent
term:Primary biliary cirrhosis
definition:A liver cirrhosis characterized by chronic and slow progressive destruction of intrahepatic bile ducts. (do).
parent_id:7695
***level:4
type:child
synonym:Pbc
term:Disposition
definition:A concept that describes the origin of a chemical, its location within an organism, or its route of exposure.
parent_id:
level:1
type:parent
term:Source
definition:Natural or synthetic origin of a chemical.
parent_id:7724
*level:2
type:parent
term:Endogenous
definition:
parent_id:7735
**level:3
type:child
term:Biological
definition:A living organism (species or a higher taxonomy rank), in which a chemical can be found.
parent_id:7735
**level:3
type:parent
term:Microbe
definition:A living organism that is of a microscopic scale, either single-celled or a cluster of cells.
parent_id:7736
***level:4
type:parent
term:Biological location
definition:The physiological origin within an organism, including anatomical compnents, biofluids and excreta.
parent_id:7724
*level:2
type:parent
term:Biofluid and excreta
definition:A liquid, semi-solid or solid material originating in the body.
parent_id:7725
**level:3
type:parent
term:Urine
definition:Excretion in liquid state processed by the kidney.
parent_id:7731
***level:4
type:child
term:Blood
definition:A liquid tissue with the primary function of transporting oxygen and carbon dioxide (nci). it supplies the tissues with nutrients, removes waste products, and contains various components of the immune system defending the body against infection.
parent_id:7731
***level:4
type:child
term:Subcellular
definition:An anatomical organizational level including a component within a biological cell .
parent_id:7725
**level:3
type:parent
term:Cytoplasm
definition:The portion of the cell contained within the plasma membrane but excluding the nucleus.
parent_id:7730
***level:4
type:child
synonym:Cytoplasma
term:Mitochondria
definition:
parent_id:7730
***level:4
type:child
term:Process
definition:Biological or chemical events, or a series thereof, leading to a known function or end-product.
parent_id:
level:1
type:parent
term:Naturally occurring process
definition:Naturally-occurring molecular events or a series thereof, leading to a known function or end-product.
parent_id:7659
*level:2
type:parent
term:Biological process
definition:Biological or chemical events or a series thereof, leading to a known function or end-product within an organism.
parent_id:7660
**level:3
type:parent
term:Biochemical pathway
definition:A linked series of chemical reactions that occur in a defined order within or between organism cells, and lead to a known function or end product.
parent_id:7661
***level:4
type:parent
term:Role
definition:The purpose or function assumed by a chemical, either naturally or as intended by humans .
parent_id:
level:1
type:parent
term:Industrial application
definition:The assumed function of a chemical utilized by human.
parent_id:7671
*level:2
type:parent
term:Food and nutrition
definition:A chemical purpose related to manufacturing of food and nutrition products.
parent_id:7678
**level:3
type:parent
term:Flavoring agent
definition:
parent_id:7687
***level:4
type:child
实验性质
1:
kind:water_solubility
value:112 mg/mL
source:
2:
kind:melting_point
value:154 °C
source:
预测性质
1:
kind:solubility
value:0.93 g/L
source:ALOGPS
2:
kind:logp
value:1.9
source:ChemAxon
3:
kind:pka_strongest_acidic
value:3.33
source:ChemAxon
4:
kind:pka_strongest_basic
value:-9.8
source:ChemAxon
5:
kind:iupac
value:2-oxo-3-phenylpropanoic acid
source:ChemAxon
6:
kind:average_mass
value:164.158
source:ChemAxon
7:
kind:mono_mass
value:164.047344122
source:ChemAxon
8:
kind:smiles
value:OC(=O)C(=O)CC1=CC=CC=C1
source:ChemAxon
9:
kind:formula
value:C9H8O3
source:ChemAxon
10:
kind:inchi
value:InChI=1S/C9H8O3/c10-8(9(11)12)6-7-4-2-1-3-5-7/h1-5H,6H2,(H,11,12)
source:ChemAxon
11:
kind:inchikey
value:BTNMPGBKDVTSJY-UHFFFAOYSA-N
source:ChemAxon
12:
kind:polar_surface_area
value:54.37
source:ChemAxon
13:
kind:refractivity
value:42.71
source:ChemAxon
14:
kind:polarizability
value:15.75
source:ChemAxon
15:
kind:rotatable_bond_count
value:3
source:ChemAxon
16:
kind:acceptor_count
value:3
source:ChemAxon
17:
kind:donor_count
value:1
source:ChemAxon
18:
kind:physiological_charge
value:-1
source:ChemAxon
19:
kind:formal_charge
value:0
source:ChemAxon
20:
kind:number_of_rings
value:1
source:ChemAxon
21:
kind:bioavailability
value:Yes
source:ChemAxon
22:
kind:rule_of_five
value:Yes
source:ChemAxon
23:
kind:ghose_filter
value:Yes
source:ChemAxon
24:
kind:veber_rule
value:No
source:ChemAxon
25:
kind:mddr_like_rule
value:No
source:ChemAxon
生物学性质
1:
cellular:(1):Cytoplasm
cellular:(2):Mitochondria
2:
biospecimen:(1):Blood
biospecimen:(2):Feces
biospecimen:(3):Urine
3:
4:
[1]:
name:Phenylalanine and Tyrosine Metabolism
smpdb_id:SMP00008
kegg_map_id:map00360
[2]:
name:Phenylketonuria
smpdb_id:SMP00206
kegg_map_id:
[3]:
name:Tyrosinemia Type 2 (or Richner-Hanhart syndrome)
smpdb_id:SMP00369
kegg_map_id:
[4]:
name:Tyrosinemia Type 3 (TYRO3)
smpdb_id:SMP00370
kegg_map_id:
正常浓度
1:
biospecimen:Blood
concentration_value:
concentration_units:
subject_age:Adult (>18 years old)
subject_sex:Both
subject_condition:Normal
2:
biospecimen:Blood
concentration_value:0.5 +/- 0.1
concentration_units:uM
subject_age:Adult (>18 years old)
subject_sex:Both
subject_condition:Normal
3:
biospecimen:Blood
concentration_value:5
concentration_units:uM
subject_age:Children (1 - 13 years old)
subject_sex:Both
subject_condition:Normal
4:
biospecimen:Feces
concentration_value:
concentration_units:
subject_age:Adult (>18 years old)
subject_sex:Both
subject_condition:Normal
5:
biospecimen:Feces
concentration_value:
concentration_units:
subject_age:Adult (>18 years old)
subject_sex:Both
subject_condition:Normal
6:
biospecimen:Urine
concentration_value:0.17 (0.05-0.67)
concentration_units:umol/mmol creatinine
subject_age:Adult (>18 years old)
subject_sex:Male
subject_condition:Normal
7:
biospecimen:Urine
concentration_value:0.24 (0.10-0.76)
concentration_units:umol/mmol creatinine
subject_age:Adult (>18 years old)
subject_sex:Female
subject_condition:Normal
8:
biospecimen:Urine
concentration_value:
concentration_units:
subject_age:Adult (>18 years old)
subject_sex:Both
subject_condition:Normal
9:
biospecimen:Urine
concentration_value:0.00064 +/- 0.000544
concentration_units:umol/mmol creatinine
subject_age:Newborn (0-30 days old)
subject_sex:Not Specified
subject_condition:Normal
10:
biospecimen:Urine
concentration_value:<2
concentration_units:umol/mmol creatinine
subject_age:Children (1 - 18 years old)
subject_sex:Both
subject_condition:Normal
11:
biospecimen:Urine
concentration_value:0.138 +/- 0.207
concentration_units:umol/mmol creatinine
subject_age:Infant (0-1 year old)
subject_sex:Both
subject_condition:Normal
12:
biospecimen:Urine
concentration_value:0.207 +/- 0.275
concentration_units:umol/mmol creatinine
subject_age:Infant (0-1 year old)
subject_sex:Both
subject_condition:Normal
13:
biospecimen:Urine
concentration_value:0.207 +/- 0.344
concentration_units:umol/mmol creatinine
subject_age:Infant (0-1 year old)
subject_sex:Both
subject_condition:Normal
14:
biospecimen:Urine
concentration_value:0.25 +/- 0.43
concentration_units:umol/mmol creatinine
subject_age:Infant (0-1 year old)
subject_sex:Both
subject_condition:Normal
异常浓度
1:
biospecimen:Blood
concentration_value:50.0 (40.0-60.0)
concentration_units:uM
patient_age:Newborn (0-30 days old)
patient_sex:Both
patient_information:Phenylketonuria
2:
biospecimen:Blood
concentration_value:1.4 +/- 0.2
concentration_units:uM
patient_age:Adult (>18 years old)
patient_sex:Both
patient_information:Primary biliary cirrhosis
3:
biospecimen:Urine
concentration_value:7796.386
concentration_units:umol/mmol creatinine
patient_age:Infant (0-1 year old)
patient_sex:Male
patient_information:Phenylketonuria
4:
biospecimen:Urine
concentration_value:31047.8
concentration_units:umol/mmol creatinine
patient_age:Children (1 - 13 years old)
patient_sex:Male
patient_information:Phenylketonuria
5:
biospecimen:Urine
concentration_value:255.518 +/- 250.00780
concentration_units:umol/mmol creatinine
patient_age:Children (1-13 years old)
patient_sex:Both
patient_information:Phenylketonuria
6:
biospecimen:Urine
concentration_value:735.560 +/- 528.253
concentration_units:umol/mmol creatinine
patient_age:Children (1-13 years old)
patient_sex:Both
patient_information:Phenylketonuria
7:
biospecimen:Urine
concentration_value:882.948 +/- 741.0699
concentration_units:umol/mmol creatinine
patient_age:Children (1-13 years old)
patient_sex:Both
patient_information:Phenylketonuria
8:
biospecimen:Urine
concentration_value:146.6 +/- 244.6
concentration_units:umol/mmol creatinine
patient_age:Newborn (0-30 days old)
patient_sex:Both
patient_information:Phenylketonuria
疾病参考
1:
name:Phenylketonuria
omim_id:261600
[1]:
reference_text:Perry TL, Hansen S: Cystinylglycine in plasma: diagnostic relevance for pyroglutamic acidemia, homocystinuria, and phenylketonuria. Clin Chim Acta. 1981 Nov 25;117(1):7-12.
pubmed_id:7333014
[2]:
reference_text: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_id:2091926
[3]:
reference_text:Deng C, Shang C, Hu Y, Zhang X: Rapid diagnosis of phenylketonuria and other aminoacidemias by quantitative analysis of amino acids in neonatal blood spots by gas chromatography-mass spectrometry. J Chromatogr B Analyt Technol Biomed Life Sci. 2002 Jul 25;775(1):115-20.
pubmed_id:12101068
[4]:
reference_text:Elvers LH, Loeber JG, Dhondt JL, Fukushi M, Hannon WH, Torresani T, Webster D: First ISNS Reference Preparation for Neonatal Screening for thyrotropin, phenylalanine and 17alpha-hydroxyprogesterone in blood spots. J Inherit Metab Dis. 2007 Aug;30(4):609. Epub 2007 Jun 14.
pubmed_id:17574536
[5]:
reference_text: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_id:6790852
[6]:
reference_text:Peck H, Pollitt RJ: The occurrence of gamma-glutamylphenylalanine in the urine of newborn phenylketonurics. Clin Chim Acta. 1979 Jun 15;94(3):237-40.
pubmed_id:466810
[7]:
reference_text:Clemens PC, Schunemann MH, Hoffmann GF, Kohlschutter A: Plasma concentrations of phenyllactic acid in phenylketonuria. J Inherit Metab Dis. 1990;13(2):227-8.
pubmed_id:2116554
[8]:
reference_text:Gronwald W, Klein MS, Kaspar H, Fagerer SR, Nurnberger N, Dettmer K, Bertsch T, Oefner PJ: Urinary metabolite quantification employing 2D NMR spectroscopy. Anal Chem. 2008 Dec 1;80(23):9288-97. doi: 10.1021/ac801627c.
pubmed_id:19551947
[9]:
reference_text:Jansen RS, Addie R, Merkx R, Fish A, Mahakena S, Bleijerveld OB, Altelaar M, IJlst L, Wanders RJ, Borst P, van de Wetering K: N-lactoyl-amino acids are ubiquitous metabolites that originate from CNDP2-mediated reverse proteolysis of lactate and amino acids. Proc Natl Acad Sci U S A. 2015 May 26;112(21):6601-6. doi: 10.1073/pnas.1424638112. Epub 2015 May 11.
pubmed_id:25964343
[10]:
reference_text:Swarna M, Jyothy A, Usha Rani P, Reddy PP: Amino acid disorders in mental retardation: a two-decade study from Andhra Pradesh. Biochem Genet. 2004 Apr;42(3-4):85-98.
pubmed_id:15168722
[11]:
reference_text:Rampini S, Vollmin JA, Bosshard HR, Muller M, Curtius HC: Aromatic acids in urine of healthy infants, persistent hyperphenylalaninemia, and phenylketonuria, before and after phenylalanine load. Pediatr Res. 1974 Jul;8(7):704-9.
pubmed_id:4837567
[12]:
reference_text:MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de)
pubmed_id:
2:
name:Primary biliary cirrhosis
omim_id:109720
[1]:
reference_text:Nikkila K, Hockerstedt K, Miettinen TA: High cholestanol and low campesterol-to-sitosterol ratio in serum of patients with primary biliary cirrhosis before liver transplantation. Hepatology. 1991 Apr;13(4):663-9.
pubmed_id:2010161
[2]:
reference_text:Braganza JM, Day JP: Serum octadeca-9-11 dienoic acid concentrations in primary biliary cirrhosis. Lancet. 1987 Apr 25;1(8539):987.
pubmed_id:2882386
[3]:
reference_text: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_id:8216688
[4]:
reference_text:Batta AK, Arora R, Salen G, Tint GS, Eskreis D, Katz S: Characterization of serum and urinary bile acids in patients with primary biliary cirrhosis by gas-liquid chromatography-mass spectrometry: effect of ursodeoxycholic acid treatment. J Lipid Res. 1989 Dec;30(12):1953-62.
pubmed_id:2621422
维基百科编号
Phenylpyruvic_acid
代谢途径的数据库编号
PHENYL-PYRUVATE
综合参考
Li, Hongbin; Luo, Yuzhong. Preparation of phenyl-pyruvic acid by dicarbonylation of benzyl halide. Faming Zhuanli Shenqing Gongkai Shuomingshu (1996), 5 pp.
一般参考
1:
protein_accession:HMDBP00100
name:Macrophage migration inhibitory factor
uniprot_id:P14174
gene_name:MIF
protein_type:Enzyme
2:
protein_accession:HMDBP00277
name:Tyrosine aminotransferase
uniprot_id:P17735
gene_name:TAT
protein_type:Enzyme
3:
protein_accession:HMDBP00283
name:Aspartate aminotransferase, cytoplasmic
uniprot_id:P17174
gene_name:GOT1
protein_type:Enzyme
4:
protein_accession:HMDBP00284
name:Aspartate aminotransferase, mitochondrial
uniprot_id:P00505
gene_name:GOT2
protein_type:Enzyme
5:
protein_accession:HMDBP00464
name:Kynurenine--oxoglutarate transaminase 1
uniprot_id:Q16773
gene_name:CCBL1
protein_type:Enzyme
6:
protein_accession:HMDBP00483
name:UDP-glucuronosyltransferase 1-1
uniprot_id:P22309
gene_name:UGT1A1
protein_type:Enzyme
7:
protein_accession:HMDBP00841
name:4-hydroxyphenylpyruvate dioxygenase
uniprot_id:P32754
gene_name:HPD
protein_type:Unknown
8:
protein_accession:HMDBP08281
name:L-amino-acid oxidase
uniprot_id:Q96RQ9
gene_name:IL4I1
protein_type:Unknown
蛋白质结合
1:
reference_text: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_id:2026685
2:
reference_text: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_id:9869358
3:
reference_text: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_id:14708889
4:
reference_text:Lasala JM, Coscia CJ: Accumulation of a tetrahydroisoquinoline in phenylketonuria. Science. 1979 Jan 19;203(4377):283-4.
pubmed_id:153583
5:
reference_text: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_id:719897
6:
reference_text:Michals K, Matalon R: Phenylalanine metabolites, attention span and hyperactivity. Am J Clin Nutr. 1985 Aug;42(2):361-5.
pubmed_id:4025205
7:
reference_text: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_id:2291475
8:
reference_text:Nierop Groot MN, de Bont JAM: Conversion of phenylalanine to benzaldehyde initiated by an aminotransferase in lactobacillus plantarum Appl Environ Microbiol. 1998 Aug;64(8):3009-13.
pubmed_id:9687465
9:
reference_text:Yannai, Shmuel. (2004) Dictionary of food compounds with CD-ROM: Additives, flavors, and ingredients. Boca Raton: Chapman & Hall/CRC.