3-Hydroxyglutaric acid (BioDeep_00000018489)
Secondary id: BioDeep_00000406088
human metabolite PANOMIX_OTCML-2023 Endogenous blood metabolite
代谢物信息卡片
3-Hydroxypentanedioic acid
化学式: C5H8O5 (148.0371718)
中文名称: 3-羟基戊二酸
谱图信息:
最多检出来源 Homo sapiens(blood) 0.39%
分子结构信息
SMILES: C(C(CC(=O)O)O)C(=O)O
InChI: InChI=1S/C5H8O5/c6-3(1-4(7)8)2-5(9)10/h3,6H,1-2H2,(H,7,8)(H,9,10)
描述信息
3-Hydroxyglutaric acid is a member of the class of compounds known as dicarboxylic acids and derivatives. These are organic compounds containing exactly two carboxylic acid groups. 3-Hydroxyglutaric acid is soluble (in water) and a weakly acidic compound (based on its pKa). When present in sufficiently high levels, 3-hydroxyglutaric 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 3-hydroxyglutaric acid are associated with glutaric aciduria type I (glutaric acidemia type I, glutaryl-CoA dehydrogenase deficiency, GA1, or GAT1). GA1 is an inherited disorder in which the body is unable to completely break down the amino acids lysine, hydroxylysine, and tryptophan due to a deficiency of mitochondrial glutaryl-CoA dehydrogenase (EC 1.3.99.7, GCDH). Excessive levels of their intermediate breakdown products (e.g. glutaric acid, glutaryl-CoA, 3-hydroxyglutaric acid, glutaconic acid) can accumulate and cause damage to the brain (and also other organs), but particularly the basal ganglia. GA1 is associated with a risk for intracranial and retinal hemorrhage, and non-specific white matter changes. Babies with glutaric acidemia type I are often born with unusually large heads (macrocephaly). Other symptoms include spasticity (increased muscle tone/stiffness) and dystonia (involuntary muscle contractions resulting in abnormal movement or posture), but many affected individuals are asymptomatic. Seizures and coma (encephalopathy) are rare. GA1 also causes secondary carnitine deficiency because 3-hydroxyglutaric acid, like other organic acids, is detoxified by carnitine.
3-Hydroxyglutaric acid is a key metabolite in glutaryl co-enzyme A dehydrogenase deficiency, and is considered to be a potential neurotoxin. The urine level of 3-Hydroxyglutaric acid is elevated in Glutaric Aciduria Type I (glutaryl-CoA dehydrogenase deficiency) patients. (PMID 16573641) [HMDB]
3-Hydroxyglutaric acid is a glutaric acid derivative.
同义名列表
13 个代谢物同义名
3-Hydroxypentanedioic acid; beta-Hydroxyglutaric acid; 2,4-Dideoxypentaric acid; 3-Hydroxy-glutaric acid; 3-Hydroxypentanedioate; Β-hydroxyglutaric acid; 3-HYDROXYGLUTARIC ACID; b-Hydroxyglutaric acid; beta-Hydroxyglutarate; 3-Hydroxy-glutarate; Β-hydroxyglutarate; b-Hydroxyglutarate; 3-Hydroxyglutarate
数据库引用编号
12 个数据库交叉引用编号
- ChEBI: CHEBI:39980
- PubChem: 181976
- HMDB: HMDB0000428
- DrugBank: DB04594
- ChEMBL: CHEMBL556937
- Wikipedia: Alpha-Hydroxyglutaric acid
- foodb: FDB022040
- chemspider: 158277
- CAS: 638-18-6
- PMhub: MS000008480
- RefMet: 3-Hydroxyglutaric acid
- medchemexpress: HY-113411
分类词条
相关代谢途径
Reactome(0)
BioCyc(0)
PlantCyc(0)
代谢反应
1 个相关的代谢反应过程信息。
Reactome(0)
BioCyc(0)
WikiPathways(1)
- Cerebral organic acidurias, including diseases:
L-2-Aminoadipic acid ⟶ 2-Oxoadipic acid
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
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
- Joshua A Dubland, Graham Sinclair, Hilary Vallance, Bojana Rakić. A simple method modification to increase separation of 2- and 3-hydroxyglutaric acid by GC-MS for clinical urine organic acids analysis.
Clinical biochemistry.
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Indian journal of pediatrics.
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Clinical chemistry.
2019 02; 65(2):351-353. doi:
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Journal of chromatography. B, Analytical technologies in the biomedical and life sciences.
2018 Oct; 1097-1098(?):101-110. doi:
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Neuroscience.
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