2-Hydroxyadipic acid (BioDeep_00000004905)
Main id: BioDeep_00000629078
Secondary id: BioDeep_00000405242
human metabolite PANOMIX_OTCML-2023 Endogenous blood metabolite
代谢物信息卡片
化学式: C6H10O5 (162.052821)
中文名称: 2-羟基己二酸
谱图信息:
最多检出来源 Homo sapiens(blood) 0.02%
分子结构信息
SMILES: C(CC(C(=O)O)O)CC(=O)O
InChI: InChI=1S/C6H10O5/c7-4(6(10)11)2-1-3-5(8)9/h4,7H,1-3H2,(H,8,9)(H,10,11)
描述信息
2-Hydroxyadipic acid is a hydroxy-dicarboxylic acid formed by the reduction of 2-ketoadipic acid. A deficiency of 2-ketoadipic dehydrogenase causes 2-ketoadipic acidemia or 2-oxoadipic acidemia (OMIM: 245130), a genetic disorder characterized by accumulation and excretion of 2-hydroxyadipic acid (with 2-ketoadipic and 2-aminoadipic) (OMMBID - The Online Metabolic and Molecular Bases of Inherited Disease, CH.95). When present in sufficiently high levels, 2-hydroxyadipic acid can act as an acidogen and a metabotoxin. An acidogen is an acidic compound that induces acidosis, which has multiple adverse effects on many organ systems. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Chronically high levels of 2-hydroxyadipic acid are associated with at least three inborn errors of metabolism including 2-oxoadipic acidemia, 2-aminoadipic aciduria, and 2-oxoadipic aciduria. 2-Hydroxyadipic acid is an organic acid. Abnormally high levels of organic acids in the blood (organic acidemia), urine (organic aciduria), the brain, and other tissues lead to general metabolic acidosis. Acidosis typically occurs when arterial pH falls below 7.35. In infants with acidosis, the initial symptoms include poor feeding, vomiting, loss of appetite, weak muscle tone (hypotonia), and lack of energy (lethargy). These can progress to heart abnormalities, kidney abnormalities, liver damage, seizures, coma, and possibly death. These are also the characteristic symptoms of the untreated IEMs mentioned above. Many affected children with organic acidemias experience intellectual disability or delayed development. In adults, acidosis or acidemia is characterized by headaches, confusion, feeling tired, tremors, sleepiness, and seizures.
2-Hydroxyadipic acid is a hydroxy-dicarboxylic acid formed by the reduction of 2-ketoadipic acid. Deficiency of 2-ketoadipic dehydrogenase causes 2-ketoadipic acidemia (OMIM 245130), a condition characterized by accumulation and excretion of 2-hydroxyadipic acid (with 2-ketoadipic and 2-aminoadipic) probably without adverse phenotypic effects.(OMMBID - The Metabolic and Molecular Bases of Inherited Disease, CH.95). A method involving derivatization and combined gas chromatography--mass spectrometry has been recently developed to separate the enantiomers of 3-hydroxyadipic acid (PMID: 3980660). It has been shown that 3-hydroxyadipic acid excreted in urine consists of at least 95\\\% of the L-enantiomer. This finding supports the hypothesis that dicarboxylic acids are degraded by ordinary beta-oxidation, and indicates that adipic acid may be converted into succinic acid. (PMID: 3980660) [HMDB]
2-Hydroxyadipic acid is an organic acid, formed by the reduction of 2-ketoadipic acid.
同义名列表
17 个代谢物同义名
2-hydroxy-Hexanedioic acid; 2,3,4-Trideoxyhexaric acid; 2-Hydroxyhexanedioic acid; alpha-Hydroxyadipic acid; DL-2-Hydroxyadipic acid; 2-Hydroxy-hexanedioate; 2,3,4-Trideoxyhexarate; 2-Hydroxyhexanedioate; 2-Hydroxy-adipic acid; α-hydroxyadipic acid; alpha-Hydroxyadipate; a-Hydroxyadipic acid; 2-hydroxyadipic acid; DL-2-Hydroxyadipate; 2-Hydroxy-adipate; a-Hydroxyadipate; 2-Hydroxyadipate
数据库引用编号
14 个数据库交叉引用编号
- ChEBI: CHEBI:17023
- KEGG: C02360
- PubChem: 193530
- HMDB: HMDB0000321
- Metlin: METLIN5310
- foodb: FDB021947
- chemspider: 167943
- CAS: 18294-85-4
- PMhub: MS000017544
- PubChem: 5406
- LipidMAPS: LMFA01170049
- NIKKAJI: J428.482H
- RefMet: 2-Hydroxyadipic acid
- medchemexpress: HY-113101
分类词条
相关代谢途径
Reactome(0)
BioCyc(0)
PlantCyc(0)
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)
2 个相关的物种来源信息
- 1080010 - Aloe africana: 10.1021/JF071110T
- 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。
文献列表
- Hui-Ming Lin, Matthew P G Barnett, Nicole C Roy, Nigel I Joyce, Shuotun Zhu, Kelly Armstrong, Nuala A Helsby, Lynnette R Ferguson, Daryl D Rowan. Metabolomic analysis identifies inflammatory and noninflammatory metabolic effects of genetic modification in a mouse model of Crohn's disease.
Journal of proteome research.
2010 Apr; 9(4):1965-75. doi:
10.1021/pr901130s
. [PMID: 20141220] - C Jakobs, A J de Grauw. A fatal case of 2-keto-, 2-hydroxy- and 2-aminoadipic aciduria: relation of organic aciduria to phenotype?.
Journal of inherited metabolic disease.
1992; 15(2):279-80. doi:
10.1007/bf01799643
. [PMID: 1527995]