D-2-Hydroxyglutaric acid (BioDeep_00000002868)

 

Secondary id: BioDeep_00001868343

human metabolite PANOMIX_OTCML-2023 Endogenous blood metabolite


代谢物信息卡片


alpha-Hydroxyglutarate, disodium salt

化学式: C5H8O5 (148.0371718)
中文名称: 2-羟基戊二酸
谱图信息: 最多检出来源 Viridiplantae(plant) 0.2%

分子结构信息

SMILES: C(CC(=O)O)C(C(=O)O)O
InChI: InChI=1S/C5H8O5/c6-3(5(9)10)1-2-4(7)8/h3,6H,1-2H2,(H,7,8)(H,9,10)/t3-/m1/s1

描述信息

In humans, D-2-hydroxyglutaric acid is formed by a hydroxyacid-oxoacid transhydrogenase whereas in bacteria it is formed by a 2-hydroxyglutarate synthase. D-2-Hydroxyglutaric acid is also formed via the normal activity of hydroxyacid-oxoacid transhydrogenase during conversion of 4-hydroxybutyrate to succinate semialdehyde. The compound can be converted to alpha-ketoglutaric acid through the action of a 2-hydroxyglutarate dehydrogenase (EC 1.1.99.2). In humans, there are two such enzymes (D2HGDH and L2HGDH). Both the D and the L stereoisomers of hydroxyglutaric acid are found in body fluids. D-2-Hydroxyglutaric acid is a biochemical hallmark of the inherited neurometabolic disorder D-2-hydroxyglutaric aciduria (OMIM: 600721) and the genetic disorder glutaric aciduria II. D-2-Hydroxyglutaric aciduria (caused by loss of D2HGDH or gain of function of IDH) is rare, with symptoms including cancer, macrocephaly, cardiomyopathy, mental retardation, hypotonia, and cortical blindness. An elevated urine level of D-2-hydroxyglutaric acid has been reported in patients with spondyloenchondrodysplasia (OMIM: 271550). D-2-Hydroxyglutaric acid can be converted to alpha-ketoglutaric acid through the action of 2-hydroxyglutarate dehydrogenase (D2HGDH). Additionally, the enzyme D-3-phosphoglycerate dehydrogenase (PHGDH) can catalyze the NADH-dependent reduction of alpha-ketoglutarate (AKG) to D-2-hydroxyglutarate (D-2HG). Nyhan et al. (1995) described 3 female patients, 2 of them sibs, who were found to have excess accumulation of D-2-hydroxyglutaric acid in the urine. The phenotype was quite variable, even among the sibs, but included mental retardation, macrocephaly with cerebral atrophy, hypotonia, seizures, and involuntary movements. One of the patients developed severe intermittent vomiting and was given a pyloromyotomy. The electroencephalogram demonstrated hypsarrhythmia. There was an increased concentration of protein in cerebrospinal fluid, an unusual finding in inborn errors of metabolism. D-2-Hydroxyglutaric acid can also be produced via gain-of-function mutations in the cytosolic and mitochondrial isoforms of isocitrate dehydrogenase (IDH). IDH is part of the TCA cycle and this compound is generated in high abundance when IDH is mutated. Since D-2-hydroxyglutaric acid is sufficiently similar in structure to 2-oxoglutarate (2OG), it is able to inhibit a range of 2OG-dependent dioxygenases, including histone lysine demethylases (KDMs) and members of the ten-eleven translocation (TET) family of 5-methylcytosine (5mC) hydroxylases. This inhibitory effect leads to alterations in the hypoxia-inducible factor (HIF)-mediated hypoxic response and alterations in gene expression through global epigenetic remodeling. The net effect is that D-2-hydroxyglutaric acid causes a cascading effect that leads genetic perturbations and malignant transformation. Depending on the circumstances, D-2-hydroxyglutaric acid can act as an oncometabolite, a neurotoxin, an acidogen, and a metabotoxin. An oncometabolite is a compound that promotes tumour growth and survival. A neurotoxin is compound that is toxic to neurons or nerual tissue. 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. As an oncometabolite, D-2-hydroxyglutaric acid is a competitive inhibitor of multiple alpha-ketoglutarate-dependent dioxygenases, including histone demethylases and the TET family of 5mC hydroxylases. As a result, high levels of 2-hydroxyglutarate lead to genome-wide histone and DNA methylation alterations, which in turn lead to mutations that ultimately cause cancer (PMID: 29038145). As a neurotoxin, D-2-hydroxyglutaric acid mediates its neurotoxicity through activation of N-methyl-D-aspartate receptors. D-2-Hydroxyglutaric acid is structurally similar to the excitatory amino acid glutamate and stimul...
Tissue accumulation of high amounts of D 2 hydroxyglutaric acid is the biochemical hallmark of the inherited neurometabolic disorder D 2 hydroxyglutaric aciduria.

同义名列表

32 个代谢物同义名

alpha-Hydroxyglutarate, disodium salt; alpha-Hydroxyglutarate, (DL)-isomer; alpha-Hydroxyglutarate, (L)-isomer; alpha-Hydroxyglutarate, (D)-isomer; delta-alpha-Hydroxyglutaric acid; (2R)-2-hydroxypentanedioic acid; (R)-2-Hydroxy-pentanedioic acid; D-α-Hydroxyglutaric acid; (R)-alpha-Hydroxyglutaric acid; 2-Hydroxy-delta-glutaric acid; delta-alpha-Hydroxyglutarate; delta-2-Hydroxyglutaric acid; (R)-2-Hydroxy-pentanedioate; (R)-a-Hydroxyglutaric acid; (R)-2-Hydroxyglutaric acid; (R)-alpha-Hydroxyglutarate; 2-Hydroxy-delta-glutarate; 2-Hydroxy-D-glutaric acid; D-a-Hydroxyglutaric acid; D-2-Hydroxyglutaric acid; (R)-Hydroxyglutaric acid; delta-2-Hydroxyglutarate; alpha-Hydroxyglutarate; (R)-a-Hydroxyglutarate; (R)-2-Hydroxyglutarate; 2-hydroxyglutaric acid; 2-Hydroxy-D-glutarate; D-2-Hydroxyglutarate; D-a-Hydroxyglutarate; (R)-Hydroxyglutarate; 2-Hydroxyglutarate; D-2-Hydroxyglutaric acid



数据库引用编号

20 个数据库交叉引用编号

分类词条

相关代谢途径

Reactome(6)

BioCyc(0)

PlantCyc(0)

代谢反应

79 个相关的代谢反应过程信息。

Reactome(71)

BioCyc(0)

WikiPathways(0)

Plant Reactome(0)

INOH(0)

PlantCyc(0)

COVID-19 Disease Map(0)

PathBank(8)

PharmGKB(0)

6 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

  • Xinting Zhu, Juan Hao, Hong Zhang, Mengyi Chi, Yaxian Wang, Jinlu Huang, Rong Xu, Zhao Xincai, Bo Xin, Xipeng Sun, Jianping Zhang, Shumin Zhou, Dongdong Cheng, Ting Yuan, Jun Ding, Shuier Zheng, Cheng Guo, Quanjun Yang. Oncometabolite D-2-hydroxyglutarate-dependent metabolic reprogramming induces skeletal muscle atrophy during cancer cachexia. Communications biology. 2023 Sep; 6(1):977. doi: 10.1038/s42003-023-05366-0. [PMID: 37741882]
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  • Rafael Teixeira Ribeiro, Andrey Vinícios Soares Carvalho, Rafael Palavro, Luz Elena Durán-Carabali, Ângela Beatris Zemniaçak, Alexandre Umpierrez Amaral, Carlos Alexandre Netto, Moacir Wajner. L-2-Hydroxyglutaric Acid Administration to Neonatal Rats Elicits Marked Neurochemical Alterations and Long-Term Neurobehavioral Disabilities Mediated by Oxidative Stress. Neurotoxicity research. 2022 Dec; ?(?):. doi: 10.1007/s12640-022-00625-0. [PMID: 36580261]
  • Gamze Tuna, Nazlı Ecem Dal-Bekar, Ali Akay, Mete Rükşen, Sertaç İşlekel, Gül Hüray İşlekel. Minimally Invasive Detection of IDH1 Mutation With Cell-Free Circulating Tumor DNA and D-2-Hydroxyglutarate, D/L-2-Hydroxyglutarate Ratio in Gliomas. Journal of neuropathology and experimental neurology. 2022 Jun; 81(7):502-510. doi: 10.1093/jnen/nlac036. [PMID: 35582888]
  • Quanjun Yang, Juan Hao, Mengyi Chi, Yaxian Wang, Jie Li, Jinlu Huang, Jianping Zhang, Mengqi Zhang, Jin Lu, Shumin Zhou, Ting Yuan, Zan Shen, Shuier Zheng, Cheng Guo. D2HGDH-mediated D2HG catabolism enhances the anti-tumor activities of CAR-T cells in an immunosuppressive microenvironment. Molecular therapy : the journal of the American Society of Gene Therapy. 2022 03; 30(3):1188-1200. doi: 10.1016/j.ymthe.2022.01.007. [PMID: 35007759]
  • Bo Wu, Zehua Li, Zepeng Kang, Chunling Ma, Haiyan Song, Fuping Lu, Zhiguang Zhu. An Enzymatic Biosensor for the Detection of D-2-Hydroxyglutaric Acid in Serum and Urine. Biosensors. 2022 Jan; 12(2):. doi: 10.3390/bios12020066. [PMID: 35200327]
  • Bernhard Strasser, Nicolas S Arango, Jason P Stockmann, Borjan Gagoski, Bijaya Thapa, Xianqi Li, Wolfgang Bogner, Philipp Moser, Julia Small, Daniel P Cahill, Tracy T Batchelor, Jorg Dietrich, Andre van der Kouwe, Jacob White, Elfar Adalsteinsson, Ovidiu C Andronesi. Improving D-2-hydroxyglutarate MR spectroscopic imaging in mutant isocitrate dehydrogenase glioma patients with multiplexed RF-receive/B0 -shim array coils at 3 T. NMR in biomedicine. 2022 01; 35(1):e4621. doi: 10.1002/nbm.4621. [PMID: 34609036]
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  • Yue Chen, Nelamangala V Nagaraja, Bin Fan, Luke Utley, Rene M Lemieux, Janeta Popovici-Muller, Lenny Dang, Hyeryun Kim, Liping Yan, Shin-San M Su, Scott A Biller, Hua Yang. Preclinical Drug Metabolism, Pharmacokinetic, and Pharmacodynamic Profiles of Ivosidenib, an Inhibitor of Mutant Isocitrate Dehydrogenase 1 for Treatment of Isocitrate Dehydrogenase 1-Mutant Malignancies. Drug metabolism and disposition: the biological fate of chemicals. 2021 10; 49(10):870-881. doi: 10.1124/dmd.120.000234. [PMID: 34321251]
  • Rafael Teixeira Ribeiro, Bianca Seminotti, Ângela Zanatta, Francine Hehn de Oliveira, Alexandre Umpierrez Amaral, Guilhian Leipnitz, Moacir Wajner. Neuronal Death, Glial Reactivity, Microglia Activation, Oxidative Stress and Bioenergetics Impairment Caused by Intracerebroventricular Administration of D-2-hydroxyglutaric Acid to Neonatal Rats. Neuroscience. 2021 09; 471(?):115-132. doi: 10.1016/j.neuroscience.2021.07.024. [PMID: 34333063]
  • C J Pirola, M Garaycoechea, D Flichman, G O Castaño, S Sookoian. Liver mitochondrial DNA damage and genetic variability of Cytochrome b - a key component of the respirasome - drive the severity of fatty liver disease. Journal of internal medicine. 2021 01; 289(1):84-96. doi: 10.1111/joim.13147. [PMID: 32634278]
  • Garrett Brinkley, Hyeyoung Nam, Eunhee Shim, Richard Kirkman, Anirban Kundu, Suman Karki, Yasaman Heidarian, Jason M Tennessen, Juan Liu, Jason W Locasale, Tao Guo, Shi Wei, Jennifer Gordetsky, Teresa L Johnson-Pais, Devin Absher, Dinesh Rakheja, Anil K Challa, Sunil Sudarshan. Teleological role of L-2-hydroxyglutarate dehydrogenase in the kidney. Disease models & mechanisms. 2020 11; 13(11):. doi: 10.1242/dmm.045898. [PMID: 32928875]
  • Martin Kery, Ioanna Papandreou. Emerging strategies to target cancer metabolism and improve radiation therapy outcomes. The British journal of radiology. 2020 Nov; 93(1115):20200067. doi: 10.1259/bjr.20200067. [PMID: 32462882]
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  • Nanxiang Xiong, Xiaofei Gao, Hongyang Zhao, Feng Cai, Fang-Cheng Zhang, Ye Yuan, Weichao Liu, Fangping He, Lauren G Zacharias, Hong Lin, Hieu S Vu, Chao Xing, Dong-Xiao Yao, Fei Chen, Benyan Luo, Wenzhi Sun, Ralph J DeBerardinis, Hao Xu, Woo-Ping Ge. Using arterial-venous analysis to characterize cancer metabolic consumption in patients. Nature communications. 2020 06; 11(1):3169. doi: 10.1038/s41467-020-16810-8. [PMID: 32576825]
  • Tong Liu, Xiang Gu, Li-Xiang Li, Ming Li, Bing Li, Xiao Cui, Xiu-Li Zuo. Microbial and metabolomic profiles in correlation with depression and anxiety co-morbidities in diarrhoea-predominant IBS patients. BMC microbiology. 2020 06; 20(1):168. doi: 10.1186/s12866-020-01841-4. [PMID: 32552668]
  • Bin Fan, Ingo K Mellinghoff, Patrick Y Wen, Maeve A Lowery, Lipika Goyal, William D Tap, Shuchi S Pandya, Erika Manyak, Liewen Jiang, Guowen Liu, Tara Nimkar, Camelia Gliser, Molly Prahl Judge, Sam Agresta, Hua Yang, David Dai. Clinical pharmacokinetics and pharmacodynamics of ivosidenib, an oral, targeted inhibitor of mutant IDH1, in patients with advanced solid tumors. Investigational new drugs. 2020 04; 38(2):433-444. doi: 10.1007/s10637-019-00771-x. [PMID: 31028664]
  • Valeria Cuccarini, Luigi Antelmi, Bianca Pollo, Rosina Paterra, Chiara Calatozzolo, Anna Nigri, Francesco DiMeco, Marica Eoli, Gaetano Finocchiaro, Greta Brenna, Irene Tramacere, Maria Grazia Bruzzone, Elena Anghileri. In vivo 2-hydroxyglutarate-proton magnetic resonance spectroscopy (3 T, PRESS technique) in treatment-naïve suspect lower-grade gliomas: feasibility and accuracy in a clinical setting. Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology. 2020 Feb; 41(2):347-355. doi: 10.1007/s10072-019-04087-9. [PMID: 31650436]
  • Tian-Xiang Wang, Jun-Yun Liang, Cheng Zhang, Yue Xiong, Kun-Liang Guan, Hai-Xin Yuan. The oncometabolite 2-hydroxyglutarate produced by mutant IDH1 sensitizes cells to ferroptosis. Cell death & disease. 2019 10; 10(10):755. doi: 10.1038/s41419-019-1984-4. [PMID: 31591388]
  • Shinji K Strain, Morris D Groves, Mark R Emmett. Differentiation of 2-hydroxyglutarate enantiomers and its lactones by gas chromatography/electron ionization tandem mass spectrometry. Rapid communications in mass spectrometry : RCM. 2019 Sep; 33(17):1401-1409. doi: 10.1002/rcm.8485. [PMID: 31148247]
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