Beta-hydroxyisovaleroylcarnitine (BioDeep_00000054535)

human metabolite Endogenous blood metabolite

代谢物信息卡片

(3R)-3-[(3-hydroxy-3-methylbutanoyl)oxy]-4-(trimethylazaniumyl)butanoate

化学式: C12H23NO5 (261.1576148)
中文名称: 3-羟基异戊酰- -肉碱
谱图信息: 最多检出来源 Homo sapiens(blood) 17.78%

Reviewed

Last reviewed on 2024-09-13.

Cite this Page

Beta-hydroxyisovaleroylcarnitine. BioDeep Database v3. PANOMIX ltd, a top metabolomics service provider from China. https://query.biodeep.cn/s/beta-hydroxyisovaleroylcarnitine (retrieved 2024-11-22) (BioDeep RN: BioDeep_00000054535). Licensed under the Attribution-Noncommercial 4.0 International License (CC BY-NC 4.0).

分子结构信息

SMILES: CC(C)(O)CC(=O)O[C@H](CC([O-])=O)C[N+](C)(C)C
InChI: InChI=1S/C12H23NO5/c1-12(2,17)7-11(16)18-9(6-10(14)15)8-13(3,4)5/h9,17H,6-8H2,1-5H3/t9-/m1/s1

描述信息

3-Hydroxyisovalerylcarnitine is structual derivative of 3-hydroxyisovaleric acid and carnitine. Due to its increased concentration in blood, 3-hydroxyisovalerylcarnitine (C5OH-I) is an important indicator for the diagnosis of organic acidemias in newborns (PMID: 18088573). Analysis of acylcarnitines by tandem mass spectrometry (MS/MS) has recently been used to screen newborns for organic acidemias and fatty acid oxidation defects (PMID: 7494654, 9216448, 11427446, 12127323, 14578311). These diseases cause the accumulation of acyl-CoA, which is esterified to acylcarnitine by carnitine acyltransferase. Acylcarnitine is then eliminated in the urine, thus acylcarnitine concentration serves as an excellent indicator for these diseases (PMID: 6361812). 3-Hydroxyisovalerylcarnitine (C5OH-I) is an indicator for diagnoses of multiple carboxylase deficiency (MCD), 3-methylcrotonyl-CoA carboxylase deficiency and 3-hydroxy-3-methylglutaryl-CoA lyase deficiency (PMID: 18088573), which are inborn errors of metabolism.

同义名列表

3 个代谢物同义名

(3R)-3-[(3-hydroxy-3-methylbutanoyl)oxy]-4-(trimethylazaniumyl)butanoate; (3R)-3-[(3-hydroxy-3-methylbutanoyl)oxy]-4-(trimethylammonio)butanoate; 3-hydroxyisovalerylcarnitine

数据库引用编号

2 个数据库交叉引用编号

HMDB: HMDB0061189
CAS: 99159-87-2

分类词条

代谢反应

0 个相关的代谢反应过程信息。

Reactome(0)

BioCyc(0)

WikiPathways(0)

Plant Reactome(0)

INOH(0)

PlantCyc(0)

COVID-19 Disease Map(0)

PathBank(0)

PharmGKB(0)

1 个相关的物种来源信息

9606 - Homo sapiens: -

在这里通过桑基图来展示出与当前的这个代谢物在我们的BioDeep知识库中具有相关联信息的其他代谢物。在这里进行关联的信息来源主要有：

PubMed: 来源于PubMed文献库中的文献信息，我们通过自然语言数据挖掘得到的在同一篇文献中被同时提及的相关代谢物列表，这个列表按照代谢物同时出现的文献数量降序排序，取前10个代谢物作为相关研究中关联性很高的代谢物集合展示在桑基图中。
NCBI Taxonomy: 通过文献数据挖掘，得到的代谢物物种来源信息关联。这个关联信息同样按照出现的次数降序排序，取前10个代谢物作为高关联度的代谢物集合展示在桑吉图上。
Chemical Taxonomy: 在物质分类上处于同一个分类集合中的其他代谢物
Chemical Reaction: 在化学反应过程中，存在为当前代谢物相关联的生化反应过程中的反应底物或者反应产物的关联代谢物信息。

点击图上的相关代谢物的名称，可以跳转到相关代谢物的信息页面。