Version 2.80

Status Information

Status
DISCOURAGED
Comment
No units are associated with this term - cannot verify validity

Part Descriptions

LP15794-8   Phenylpyruvate
Phenylpyruvic acid is a pyruvic acid derivative. Copyright Text is available under the Creative Commons Attribution/Share-Alike License. See http://creativecommons.org/licenses/by-sa/3.0/ for details. Source: Wikipedia , Wikipedia

LP15794-8   Phenylpyruvate
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. Copyright Copyright ©2005-2009 Genome Alberta (Reference to original publication: Wishart DS, Knox C, Guo AC, et al. HMDB: a knowledgebase for the human metabolome. Nucleic Acids Res. 2009 37(Database issue):D603-610.) Source: Human Metabolome Database , Human Metabolome Database

Fully-Specified Name

Component
Phenylpyruvate
Property
MCnc
Time
Pt
System
Bld
Scale
Qn
Method

Additional Names

Long Common Name
Phenylpyruvate [Mass/volume] in Blood
Short Name
Phenylpyruvate Bld-mCnc
Display Name
Phenylpyruvate (Bld) [Mass/Vol]
Consumer Name Alpha Get Info
Phenylpyruvate, Blood

Basic Attributes

Class
CHEM
Type
Laboratory
First Released
Version 1.0
Last Updated
Version 2.44 (MIN)
Order vs. Observation
Both

Member of these Groups Get Info

LOINC Group Group Name
LG10847-8 Phenylpyruvate|MCnc|Pt|ANYBldSerPl

Language Variants Get Info

Tag Language Translation
el-GR Greek (Greece) Φαινυλοπυρουβικό οξύ:MCnc:Pt:Αίμα:Qn:
Synonyms: MCnc
es-AR Spanish (Argentina) fenilpiruvato:concentración de masa:punto en el tiempo:sangre:cuantitativo:
es-ES Spanish (Spain) Fenilpiruvato:Concentración de masa:Punto temporal:Sangre:Qn:
Synonyms: Cuantitativo
es-MX Spanish (Mexico) Fenilpiruvato:Concentración de masa:Punto temporal:Sangre:Cuantitativo:
et-EE Estonian (Estonia) Fenüülpüruvaat:MCnc:Pt:B:Qn:
Synonyms: Juhuslik Kvantitatiivne Veri
fr-CA French (Canada) Phénylpyruvate:Concentration de masse:Temps ponctuel:Sang:Quantitatif:
fr-BE French (Belgium) Phénylpyruvate:Concentration de masse:Temps ponctuel:Sang:Quantitatif:
it-IT Italian (Italy) Fenilpiruvato:MCnc:Pt:Sangue:Qn:
Synonyms: Chimica Concentrazione di Massa Punto nel tempo (episodio) Sangue
ko-KR Korean (Korea, Republic Of) 페닐피루브산염:질량농도:검사시점:전혈:정량:
nl-NL Dutch (Netherlands) fenylpyruvaat:massa/volume:moment:bloed:kwantitatief:
pl-PL Polish (Poland) Kwas fenylopirogronowy:stężenie masowe:punkt w czasie:krew:ilościowy:
pt-BR Portuguese (Brazil) Fenilpiruvato:MCnc:Pt:SgTotal:Qn:
Synonyms: Phenylpyruvic acid; Mass concentration; Level; Point in time; Random; Blood; WB; Whole blood; Quantitative; QNT; Quant; Quan; Chemistry
ru-RU Russian (Russian Federation) Фенилпируват:МассКонц:ТчкВрм:Кр:Колич:
Synonyms: Количественный Кровь Массовая концентрация Точка во времени;Момент
tr-TR Turkish (Turkey) Fenilpiruvat:KütlKons:Zmlı:Kan:Kant:
zh-CN Chinese (China) 苯丙酮酸:质量浓度:时间点:全血:定量型:
Synonyms: 化学;化学检验项目;化学检验项目类;化学类;化学试验;非刺激耐受型化学检验项目;非刺激耐受型化学检验项目类;非刺激耐受型化学试验;非刺激耐受型化学试验类 可用数量表示的;定量性;数值型;数量型;连续数值型标尺 时刻;随机;随意;瞬间 水平;浓度;质量浓度(单位体积) 血;血液

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