Methylsuccinate (BioDeep_00000018328)

Main id: BioDeep_00000628747

 

human metabolite PANOMIX_OTCML-2023 Endogenous blood metabolite


代谢物信息卡片


(-)-(S)-alpha-Methylsuccinic acid

化学式: C5H8O4 (132.0422568)
中文名称: 2-甲基琥珀酸, (S)-(-)-甲基琥珀酸, 甲基琥珀酸
谱图信息: 最多检出来源 () 0%

Reviewed

Last reviewed on 2024-09-14.

Cite this Page

Methylsuccinate. BioDeep Database v3. PANOMIX ltd, a top metabolomics service provider from China. https://query.biodeep.cn/s/methylsuccinate (retrieved 2024-09-18) (BioDeep RN: BioDeep_00000018328). Licensed under the Attribution-Noncommercial 4.0 International License (CC BY-NC 4.0).

分子结构信息

SMILES: CC(CC(=O)O)C(=O)O
InChI: InChI=1S/C5H8O4/c1-3(5(8)9)2-4(6)7/h3H,2H2,1H3,(H,6,7)(H,8,9)

描述信息

Methylsuccinic acid (CAS: 498-21-5) is a normal metabolite found in human fluids. Increased urinary levels of methylsuccinic acid (together with ethylmalonic acid) are the main biochemical measurable features in ethylmalonic encephalopathy (OMIM: 602473), a rare metabolic disorder with an autosomal recessive mode of inheritance that is clinically characterized by neuromotor delay, hyperlactic acidemia, recurrent petechiae, orthostatic acrocyanosis, and chronic diarrhea (PMID:12382164). The underlying biochemical defect involves isoleucine catabolism (PMID:9667231). Methylsuccinic acid levels were found to have decreased in the urine of animals under D-serine-induced nephrotoxicity (D-serine causes selective necrosis of the proximal straight tubules in the rat kidney) (PMID:15596249). Moreover, methylsuccinic acid is found to be associated with ethylmalonic encephalopathy, isovaleric acidemia, and medium-chain acyl-CoA dehydrogenase deficiency, which are also inborn errors of metabolism.
Methylsuccinic acid is a normal metabolite found in human fluids. Increased urinary levels of Methylsuccinic acid (together with ethylmalonic acid) are the main biochemical measurable features in ethylmalonic encephalopathy (OMIM 602473 ), a rare metabolic disorder with an autosomal recessive mode of inheritance that is clinically characterized by neuromotor delay, hyperlactic acidemia, recurrent petechiae, orthostatic acrocyanosis, and chronic diarrhea. (PMID 12382164) T he underlying biochemical defect involves isoleucine catabolism. (PMID 9667231)
2-Methylsuccinic acid is a normal metabolite in human fluids and the main biochemical measurable features in ethylmalonic encephalopathy.

同义名列表

30 个代谢物同义名

(-)-(S)-alpha-Methylsuccinic acid; (-)-(2S)-Methylbutanedioic acid; (S)-alpha-Methylsuccinic acid; (-)-alpha-Methylsuccinic acid; (2S)-2-methylbutanedioic acid; (S)-(-)-2-Methylsuccinic acid; 2-Methylbutane-1,4-dioic acid; (-)-(S)-Α-methylsuccinic acid; 1,2-Propanedicarboxylic acid; (S)-2-Methylbutanedioic acid; (S)-(-)-Methylsuccinic acid; (2S)-Methylbutanedioic acid; (S)-2-Methylsuccinic acid; (-)-Α-methylsuccinic acid; (-)-2-Methylsuccinic acid; (S)-Α-methylsuccinic acid; alpha-Methylsuccinic acid; 2-Methylbutanedioic acid; (S)-2-Methylbutanedioate; (S)-Methylsuccinic acid; (-)-Methylsuccinic acid; Methylbutanedioic acid; 2-Methylsuccinic acid; Α-methylsuccinic acid; (S)-Methylsuccinate; Methylsuccinic acid; Pyrotartaric acid; UNII:H1547KG7UZ; Methylsuccinate; Methylsuccinic acid



数据库引用编号

13 个数据库交叉引用编号

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)

4 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

  • Kuan-Wei Peng, Allison Klotz, Arcan Guven, Kayleigh Gray, Tracey Friss, Shobha Ravipaty, Rangaprasad Sarangarajan, Vladimir Tolstikov, Mark D Kellogg, Niven R Narain, Michael A Kiebish. Multiplexed LC-MS/MS analysis of methylsuccinic acid, ethylmalonic acid, and glutaric acid in plasma and urine. Analytical biochemistry. 2022 05; 645(?):114604. doi: 10.1016/j.ab.2022.114604. [PMID: 35217005]
  • Jakub Cedrowski, Kajetan Dąbrowa, Paweł Przybylski, Agnieszka Krogul-Sobczak, Grzegorz Litwinienko. Antioxidant activity of two edible isothiocyanates: Sulforaphane and erucin is due to their thermal decomposition to sulfenic acids and methylsulfinyl radicals. Food chemistry. 2021 Aug; 353(?):129213. doi: 10.1016/j.foodchem.2021.129213. [PMID: 33774519]
  • Irene De Biase, Marzia Pasquali, Alexander Asamoah. Unusual Metabolites in a Patient with Isovaleric Acidemia. Clinical chemistry. 2019 04; 65(4):595-597. doi: 10.1373/clinchem.2018.300558. [PMID: 30923063]
  • Xueqing Hu, Jiaying Shen, Xueyan Pu, Ningning Zheng, Zhongping Deng, Zean Zhang, Houkai Li. Urinary Time- or Dose-Dependent Metabolic Biomarkers of Aristolochic Acid-Induced Nephrotoxicity in Rats. Toxicological sciences : an official journal of the Society of Toxicology. 2017 03; 156(1):123-132. doi: 10.1093/toxsci/kfw244. [PMID: 28115647]
  • Yoo-Mi Kim, Chong-Kun Cheon, Kyung-Hee Park, SungWon Park, Gu-Hwan Kim, Han-Wook Yoo, Kyung-A Lee, Jung Min Ko. Novel and Recurrent ACADS Mutations and Clinical Manifestations Observed in Korean Patients with Short-chain Acyl-coenzyme a Dehydrogenase Deficiency. Annals of clinical and laboratory science. 2016 Jul; 46(4):360-6. doi: . [PMID: 27466294]
  • Lindsay Tomlinson, Mark A Tirmenstein, Evan B Janovitz, Nelly Aranibar, Karl-Heinz Ott, John C Kozlosky, Laura M Patrone, William E Achanzar, Karen A Augustine, Kimberly C Brannen, Kenneth E Carlson, Jeffrey H Charlap, Katherine M Dubrow, Liya Kang, Laura T Rosini, Julieta M Panzica-Kelly, Oliver P Flint, Frederic J Moulin, John R Megill, Haiying Zhang, Michael J Bennett, Joseph J Horvath. Cannabinoid receptor antagonist-induced striated muscle toxicity and ethylmalonic-adipic aciduria in beagle dogs. Toxicological sciences : an official journal of the Society of Toxicology. 2012 Oct; 129(2):268-79. doi: 10.1093/toxsci/kfs217. [PMID: 22821849]
  • B T Sponseller, S J Valberg, N E Schultz, H Bedford, D M Wong, K Kersh, G D Shelton. Equine multiple acyl-CoA dehydrogenase deficiency (MADD) associated with seasonal pasture myopathy in the midwestern United States. Journal of veterinary internal medicine. 2012 Jul; 26(4):1012-8. doi: 10.1111/j.1939-1676.2012.00957.x. [PMID: 22708588]
  • Natalie M Gallant, Karen Leydiker, Hao Tang, Lisa Feuchtbaum, Fred Lorey, Rebecca Puckett, Joshua L Deignan, Julie Neidich, Naghmeh Dorrani, Erica Chang, Bruce A Barshop, Stephen D Cederbaum, Jose E Abdenur, Raymond Y Wang. Biochemical, molecular, and clinical characteristics of children with short chain acyl-CoA dehydrogenase deficiency detected by newborn screening in California. Molecular genetics and metabolism. 2012 May; 106(1):55-61. doi: 10.1016/j.ymgme.2012.02.007. [PMID: 22424739]
  • C M Westermann, L Dorland, O P van Diggelen, K Schoonderwoerd, J Bierau, H R Waterham, J H van der Kolk. Decreased oxidative phosphorylation and PGAM deficiency in horses suffering from atypical myopathy associated with acquired MADD. Molecular genetics and metabolism. 2011 Nov; 104(3):273-8. doi: 10.1016/j.ymgme.2011.07.022. [PMID: 21843962]
  • A Drousiotou, I DiMeo, R Mineri, Th Georgiou, G Stylianidou, V Tiranti. Ethylmalonic encephalopathy: application of improved biochemical and molecular diagnostic approaches. Clinical genetics. 2011 Apr; 79(4):385-90. doi: 10.1111/j.1399-0004.2010.01457.x. [PMID: 20528888]
  • A M Hall, M Campanella, A Loesch, M R Duchen, R J Unwin. Albumin uptake in OK cells exposed to rotenone: a model for studying the effects of mitochondrial dysfunction on endocytosis in the proximal tubule?. Nephron. Physiology. 2010; 115(2):p9-p19. doi: 10.1159/000314540. [PMID: 20484937]
  • Zuoshan Feng, Yujia Bai, Fanglin Lu, Wenshu Huang, Xinmin Li, Xiaosong Hu. Effect of asafoetida extract on growth and quality of Pleurotus ferulic. International journal of molecular sciences. 2009 Dec; 11(1):41-51. doi: 10.3390/ijms11010041. [PMID: 20162000]
  • D T Loots. Abnormal tricarboxylic acid cycle metabolites in isovaleric acidaemia. Journal of inherited metabolic disease. 2009 Jun; 32(3):403-11. doi: 10.1007/s10545-009-1071-6. [PMID: 19343532]
  • C M Westermann, L Dorland, D M Votion, M G M de Sain-van der Velden, I D Wijnberg, R J A Wanders, W G M Spliet, N Testerink, R Berger, J P N Ruiter, J H van der Kolk. Acquired multiple Acyl-CoA dehydrogenase deficiency in 10 horses with atypical myopathy. Neuromuscular disorders : NMD. 2008 May; 18(5):355-64. doi: 10.1016/j.nmd.2008.02.007. [PMID: 18406615]
  • C M Westermann, M G M de Sain-van der Velden, J H van der Kolk, R Berger, I D Wijnberg, J P Koeman, R J A Wanders, J A Lenstra, N Testerink, A B Vaandrager, C Vianey-Saban, C Acquaviva-Bourdain, L Dorland. Equine biochemical multiple acyl-CoA dehydrogenase deficiency (MADD) as a cause of rhabdomyolysis. Molecular genetics and metabolism. 2007 Aug; 91(4):362-9. doi: 10.1016/j.ymgme.2007.04.010. [PMID: 17540595]
  • M Fex, M Dekker Nitert, N Wierup, F Sundler, C Ling, H Mulder. Enhanced mitochondrial metabolism may account for the adaptation to insulin resistance in islets from C57BL/6J mice fed a high-fat diet. Diabetologia. 2007 Jan; 50(1):74-83. doi: 10.1007/s00125-006-0464-4. [PMID: 17093947]
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  • Amy G Aslamkhan, Yong-Hae Han, Xiao-Ping Yang, Rudolfs K Zalups, John B Pritchard. Human renal organic anion transporter 1-dependent uptake and toxicity of mercuric-thiol conjugates in Madin-Darby canine kidney cells. Molecular pharmacology. 2003 Mar; 63(3):590-6. doi: 10.1124/mol.63.3.590. [PMID: 12606766]
  • Salvatore Grosso, Rosa Mostardini, Maria Angela Farnetani, Massimo Molinelli, Rosario Berardi, Carlo Dionisi-Vici, Cristiano Rizzo, Guido Morgese, Paolo Balestri. Ethylmalonic encephalopathy: further clinical and neuroradiological characterization. Journal of neurology. 2002 Oct; 249(10):1446-50. doi: 10.1007/s00415-002-0880-4. [PMID: 12382164]
  • M J Nowaczyk, D C Lehotay, B A Platt, L Fisher, R Tan, H Phillips, J T Clarke. Ethylmalonic and methylsuccinic aciduria in ethylmalonic encephalopathy arise from abnormal isoleucine metabolism. Metabolism: clinical and experimental. 1998 Jul; 47(7):836-9. doi: 10.1016/s0026-0495(98)90122-6. [PMID: 9667231]
  • M J Nowaczyk, S I Blaser, J T Clarke. Central nervous system malformations in ethylmalonic encephalopathy. American journal of medical genetics. 1998 Jan; 75(3):292-6. doi: 10.1002/(sici)1096-8628(19980123)75:3<292::aid-ajmg13>3.0.co;2-k. [PMID: 9475600]
  • I A Qureshi, D Leblanc, D Cyr, R Giguère, G Mitchell. Breeding experiments to combine the X-linked sparse-fur (spf) mutation with the autosomal recessive BALB/cByJ strain: testing the biochemical phenotype of double-mutant mice as a model for ammonia: fatty acyl CoA synergism. Biochemical and biophysical research communications. 1993 Mar; 191(2):744-9. doi: 10.1006/bbrc.1993.1280. [PMID: 8461026]
  • B A Amendt, C Greene, L Sweetman, J Cloherty, V Shih, A Moon, L Teel, W J Rhead. Short-chain acyl-coenzyme A dehydrogenase deficiency. Clinical and biochemical studies in two patients. The Journal of clinical investigation. 1987 May; 79(5):1303-9. doi: 10.1172/jci112953. [PMID: 3571488]
  • K J Ullrich, G Rumrich, G Fritzsch, S Klöss. Contraluminal para-aminohippurate (PAH) transport in the proximal tubule of the rat kidney. II. Specificity: aliphatic dicarboxylic acids. Pflugers Archiv : European journal of physiology. 1987 Jan; 408(1):38-45. doi: 10.1007/bf00581838. [PMID: 3822770]