FA 5:1;O2 (BioDeep_00000628747)

Main id: BioDeep_00000017879

Secondary id: BioDeep_00000004511, BioDeep_00000014587, BioDeep_00000398348, BioDeep_00000400094, BioDeep_00000415757, BioDeep_00000594951, BioDeep_00001868828, BioDeep_00001869044

PANOMIX_OTCML-2023 BioNovoGene_Lab2019 Volatile Flavor Compounds


代谢物信息卡片


(4S)-4-hydroxy-2-ketovaleric acid;(4S)-4-hydroxy-2-oxovaleric acid;(S)-4-hydroxy-2-ketopentanoic acid

化学式: C5H8O4 (132.0422568)
中文名称: 2-甲基琥珀酸, 乙基丙二酸, 2-羟基-4-氧代-2-戊酸, 甲基丁二酸, 甲基琥珀酸
谱图信息: 最多检出来源 Homo sapiens(feces) 0.73%

分子结构信息

SMILES: C(=O)(C(=O)O)C(O)(C)C
InChI: InChI=1S/C5H8O4/c1-3(6)5(2,9)4(7)8/h9H,1-2H3,(H,7,8)

描述信息

D018377 - Neurotransmitter Agents > D018847 - Opioid Peptides
D018377 - Neurotransmitter Agents > D004399 - Dynorphins
2-Methylsuccinic acid is a normal metabolite in human fluids and the main biochemical measurable features in ethylmalonic encephalopathy.
Ethylmalonic acid is non-carcinogenic potentially toxic and associated with anorexia nervosa and malonyl-CoA decarboxylase deficiency.

同义名列表

22 个代谢物同义名

2-methyl-butanedioic acid; Methylsuccinic acid; UNII:H1547KG7UZ; FA 5:1;O2; 2-ethyl-propanedioic acid; ETHYLMALONIC ACID; 2,2-dimethyl-propanedioic acid; dimethyl-malonic acid; PENTANOIC ACID, 2-HYDROXY-4-OXO-; 2-Hydroxy-4-oxopentanoic acid; 2-hydroxy-2-methyl-3-oxobutanoic acid; 2-Acetolactate; (4S)-4-hydroxy-2-ketovaleric acid;(4S)-4-hydroxy-2-oxovaleric acid;(S)-4-hydroxy-2-ketopentanoic acid; (4S)-4-hydroxy-2-oxopentanoic acid; (S)-4-hydroxy-2-oxopentanoic acid; (2S)-2-hydroxy-2-methyl-3-oxobutanoic acid; (S)-2-Acetolactate; 3-hydroxy-3-methyl-2-oxo-butanoic acid; Pyrotartaric acid; 2-Methylsuccinic acid; Ethylmalonic acid; Methylsuccinic acid



数据库引用编号

41 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)

7 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]
  • Sarah Fogh, Graziana Dipace, Anne Bie, Maria Veiga-da-Cunha, Jakob Hansen, Margrethe Kjeldsen, Signe Mosegaard, Antonia Ribes, Niels Gregersen, Lars Aagaard, Emile Van Schaftingen, Rikke K J Olsen. Variants in the ethylmalonyl-CoA decarboxylase (ECHDC1) gene: a novel player in ethylmalonic aciduria?. Journal of inherited metabolic disease. 2021 09; 44(5):1215-1225. doi: 10.1002/jimd.12394. [PMID: 33973257]
  • Irene De Biase, Tatiana Yuzyuk, Alicia Hernandez, Alice Basinger. An Unusually High Excretion of Ethylmalonic Acid in a Patient with Multiple Acyl-CoA Dehydrogenase Deficiency. Clinical chemistry. 2021 09; 67(9):1290-1292. doi: 10.1093/clinchem/hvab096. [PMID: 34470039]
  • 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]
  • Sirun Özçelik, Nevin Öztekin, Ertuğrul Kıykım, Mehmet Şerif Cansever, Ayşe Çiğdem Aktuğlu-Zeybek. Capillary electrophoresis with capacitively coupled contactless conductivity detection for the determination of urinary ethylmalonic acid for the diagnosis of ethylmalonic aciduria. Journal of separation science. 2020 Apr; 43(7):1365-1371. doi: 10.1002/jssc.201901044. [PMID: 31958360]
  • 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]
  • Kaihui Zhang, Yan Huang, Zhongtao Gai, Yi Liu. [Clinical and genetic analysis of a case with atypical ethyl malonate encephalopathy]. Zhonghua yi xue yi chuan xue za zhi = Zhonghua yixue yichuanxue zazhi = Chinese journal of medical genetics. 2018 Oct; 35(5):694-698. doi: 10.3760/cma.j.issn.1003-9406.2018.05.017. [PMID: 30298498]
  • Xinyang Wang, Xinshu Zhao, Jing Chou, Jiaying Yu, Tongshu Yang, Liyan Liu, Fengmin Zhang. Taurine, glutamic acid and ethylmalonic acid as important metabolites for detecting human breast cancer based on the targeted metabolomics. Cancer biomarkers : section A of Disease markers. 2018; 23(2):255-268. doi: 10.3233/cbm-181500. [PMID: 30103303]
  • Roy W A Peake, Lance H Rodan. Ethylmalonic Aciduria in an Infant with Neurological and Skin Presentation. Clinical chemistry. 2017 11; 63(11):1771-1773. doi: 10.1373/clinchem.2017.279497. [PMID: 29089323]
  • Chandra Shekar R Ambati, Furong Yuan, Lutfi A Abu-Elheiga, Yiqing Zhang, Vivekananda Shetty. Identification and Quantitation of Malonic Acid Biomarkers of In-Born Error Metabolism by Targeted Metabolomics. Journal of the American Society for Mass Spectrometry. 2017 05; 28(5):929-938. doi: 10.1007/s13361-017-1631-1. [PMID: 28315235]
  • 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]
  • M Trent Herdman, Natthida Sriboonvorakul, Stije J Leopold, Sam Douthwaite, Sanjib Mohanty, M Mahtab Uddin Hassan, Richard J Maude, Hugh W F Kingston, Katherine Plewes, Prakaykaew Charunwatthana, Kamolrat Silamut, Charles J Woodrow, Aniruddha Ghose, Kesinee Chotinavich, Md Amir Hossain, M Abul Faiz, Saroj Mishra, Natchanun Leepipatpiboon, Nicholas J White, Nicholas P J Day, Joel Tarning, Arjen M Dondorp. The role of previously unmeasured organic acids in the pathogenesis of severe malaria. Critical care (London, England). 2015 Sep; 19(?):317. doi: 10.1186/s13054-015-1023-5. [PMID: 26343146]
  • Luciana Ritter, Daniele Kleemann, Fernanda Hermes Hickmann, Alexandre Umpierrez Amaral, Ângela Sitta, Moacir Wajner, César Augusto João Ribeiro. Disturbance of energy and redox homeostasis and reduction of Na+,K+-ATPase activity provoked by in vivo intracerebral administration of ethylmalonic acid to young rats. Biochimica et biophysica acta. 2015 May; 1852(5):759-67. doi: 10.1016/j.bbadis.2015.01.003. [PMID: 25583115]
  • Patrícia Fernanda Schuck, Ana Paula Milanez, Francine Felisberto, Leticia Selinger Galant, Jéssica Luca Machado, Camila Brulezi Furlanetto, Fabricia Petronilho, Felipe Dal-Pizzol, Emilio Luiz Streck, Gustavo Costa Ferreira. Brain and muscle redox imbalance elicited by acute ethylmalonic acid administration. PloS one. 2015; 10(5):e0126606. doi: 10.1371/journal.pone.0126606. [PMID: 26010931]
  • Evangelos Pavlou, Persephone Augoustides-Savvopoulou, Niels Gregersen, Dorothea Haas, Anastasia Gkampeta, Fani Athanassiadou-Piperopoulou. An infant with ethylmalonic encephalopathy masquerading as a hematologic disorder. Journal of child neurology. 2013 May; 28(5):668-71. doi: 10.1177/0883073812449070. [PMID: 22805253]
  • Imad Dweikat, Enas Naser, Nadera Damsah, Bassam Abu Libdeh, Izzeddin Bakri. Ethylmalonic encephalopathy associated with crescentic glomerulonephritis. Metabolic brain disease. 2012 Dec; 27(4):613-6. doi: 10.1007/s11011-012-9313-y. [PMID: 22584649]
  • 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]
  • Meghan M Holdorf, Heather A Owen, Sarah Rhee Lieber, Li Yuan, Nicole Adams, Carole Dabney-Smith, Christopher A Makaroff. Arabidopsis ETHE1 encodes a sulfur dioxygenase that is essential for embryo and endosperm development. Plant physiology. 2012 Sep; 160(1):226-36. doi: 10.1104/pp.112.201855. [PMID: 22786886]
  • 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]
  • Hyejin Lee, Steven E Ullrich, Ian C Burke, Joseph Yenish, Timothy C Paulitz. Interactions between the root pathogen Rhizoctonia solani AG-8 and acetolactate-synthase-inhibiting herbicides in barley. Pest management science. 2012 Jun; 68(6):845-52. doi: 10.1002/ps.2336. [PMID: 22307918]
  • 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]
  • Lewis H Ziska, David R Gealy, Martha B Tomecek, Aaron K Jackson, Howard L Black. Recent and projected increases in atmospheric CO2 concentration can enhance gene flow between wild and genetically altered rice (Oryza sativa). PloS one. 2012; 7(5):e37522. doi: 10.1371/journal.pone.0037522. [PMID: 22649533]
  • Carole L Linster, Gaëtane Noël, Vincent Stroobant, Didier Vertommen, Marie-Françoise Vincent, Guido T Bommer, Maria Veiga-da-Cunha, Emile Van Schaftingen. Ethylmalonyl-CoA decarboxylase, a new enzyme involved in metabolite proofreading. The Journal of biological chemistry. 2011 Dec; 286(50):42992-3003. doi: 10.1074/jbc.m111.281527. [PMID: 22016388]
  • 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]
  • Jerry M Green, Micheal D K Owen. Herbicide-resistant crops: utilities and limitations for herbicide-resistant weed management. Journal of agricultural and food chemistry. 2011 Jun; 59(11):5819-29. doi: 10.1021/jf101286h. [PMID: 20586458]
  • 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]
  • Zarazuela Zolkipli, Christina B Pedersen, Anne-Marie Lamhonwah, Niels Gregersen, Ingrid Tein. Vulnerability to oxidative stress in vitro in pathophysiology of mitochondrial short-chain acyl-CoA dehydrogenase deficiency: response to antioxidants. PloS one. 2011 Apr; 6(4):e17534. doi: 10.1371/journal.pone.0017534. [PMID: 21483766]
  • Annet M Bosch, Nico G G M Abeling, Lodewijk Ijlst, Hennie Knoester, W Ludo van der Pol, Alida E M Stroomer, Ronald J Wanders, Gepke Visser, Frits A Wijburg, Marinus Duran, Hans R Waterham. Brown-Vialetto-Van Laere and Fazio Londe syndrome is associated with a riboflavin transporter defect mimicking mild MADD: a new inborn error of metabolism with potential treatment. Journal of inherited metabolic disease. 2011 Feb; 34(1):159-64. doi: 10.1007/s10545-010-9242-z. [PMID: 21110228]
  • Se Hwa Kim, Hyung-Doo Park, Young Bae Sohn, Sung Won Park, Sung Yoon Cho, Suntae Ji, Su Jin Kim, Eun Wha Choi, Chi Hwa Kim, Ah-Ra Ko, Sunghee Yeau, Kyung-Hoon Paik, Dong-Kyu Jin. Mutations of ACADS gene associated with short-chain acyl-coenzyme A dehydrogenase deficiency. Annals of clinical and laboratory science. 2011; 41(1):84-8. doi: . [PMID: 21325261]
  • Ewa Jamroz, Justyna Paprocka, Dariusz Adamek, Justyna Pytel, Katarzyna Szczechowska, Natalia Grabska, Michalina Malec, Ewa Głuszkiewicz, Michał Daab, Anatolij Wodołażski. Clinical and neuropathological picture of ethylmalonic aciduria - diagnostic dilemma. Folia neuropathologica. 2011; 49(1):71-7. doi: NULL. [PMID: 21455846]
  • Eric T McDowell, Jeremy Kapteyn, Adam Schmidt, Chao Li, Jin-Ho Kang, Anne Descour, Feng Shi, Matthew Larson, Anthony Schilmiller, Lingling An, A Daniel Jones, Eran Pichersky, Carol A Soderlund, David R Gang. Comparative functional genomic analysis of Solanum glandular trichome types. Plant physiology. 2011 Jan; 155(1):524-39. doi: 10.1104/pp.110.167114. [PMID: 21098679]
  • Makoto Takei, Yosuke Ando, Wataru Saitoh, Tomoe Tanimoto, Naoki Kiyosawa, Sunao Manabe, Atsushi Sanbuissho, Osamu Okazaki, Haruo Iwabuchi, Takashi Yamoto, Klaus-Peter Adam, James E Weiel, John A Ryals, Michael V Milburn, Lining Guo. Ethylene glycol monomethyl ether-induced toxicity is mediated through the inhibition of flavoprotein dehydrogenase enzyme family. Toxicological sciences : an official journal of the Society of Toxicology. 2010 Dec; 118(2):643-52. doi: 10.1093/toxsci/kfq211. [PMID: 20616209]
  • Magalie Barth, Chris Ottolenghi, Laurence Hubert, Dominique Chrétien, Valérie Serre, Stéphanie Gobin, Stéphane Romano, Anne Vassault, Aziz Sefiani, Daniel Ricquier, Nathalie Boddaert, Michèle Brivet, Yves de Keyzer, Arnold Munnich, Marinus Duran, Daniel Rabier, Vassili Valayannopoulos, Pascale de Lonlay. Multiple sources of metabolic disturbance in ETHE1-related ethylmalonic encephalopathy. Journal of inherited metabolic disease. 2010 Dec; 33 Suppl 3(?):S443-53. doi: 10.1007/s10545-010-9227-y. [PMID: 20978941]
  • Bianca T van Maldegem, Ronald J A Wanders, Frits A Wijburg. Clinical aspects of short-chain acyl-CoA dehydrogenase deficiency. Journal of inherited metabolic disease. 2010 Oct; 33(5):507-11. doi: 10.1007/s10545-010-9080-z. [PMID: 20429031]
  • Qin Yu, Heping Han, Martin M Vila-Aiub, Stephen B Powles. AHAS herbicide resistance endowing mutations: effect on AHAS functionality and plant growth. Journal of experimental botany. 2010 Sep; 61(14):3925-34. doi: 10.1093/jxb/erq205. [PMID: 20627897]
  • Christina B Pedersen, Zarazuela Zolkipli, Søren Vang, Johan Palmfeldt, Margrethe Kjeldsen, Vibeke Stenbroen, Stinne P Schmidt, Ronald J A Wanders, Jos P N Ruiter, Flemming Wibrand, Ingrid Tein, Niels Gregersen. Antioxidant dysfunction: potential risk for neurotoxicity in ethylmalonic aciduria. Journal of inherited metabolic disease. 2010 Jun; 33(3):211-22. doi: 10.1007/s10545-010-9086-6. [PMID: 20443061]
  • Safiyh Taghavi, Daniel van der Lelie, Adam Hoffman, Yian-Biao Zhang, Michael D Walla, Jaco Vangronsveld, Lee Newman, Sébastien Monchy. Genome sequence of the plant growth promoting endophytic bacterium Enterobacter sp. 638. PLoS genetics. 2010 May; 6(5):e1000943. doi: 10.1371/journal.pgen.1000943. [PMID: 20485560]
  • Raffaella Zannolli, Sabrina Buoni, Maria Tassini, Anna De Nicola, Gianni Betti, Claudio De Felice, Alessandra Orsi, Maria Concetta Varetti, Francesco Ferrara, Mario Messina, Cosimo Giannini, Angelika Mohn, Francesco Chiarelli, Marco Liberati, Mirella Strambi, Silvia Funghini, Antonio Vivi, Ron A Wevers, Joseph Hayek. Silent increase of urinary ethylmalonic acid is an indicator of nonspecific brain dysfunction. NMR in biomedicine. 2010 May; 23(4):353-8. doi: 10.1002/nbm.1468. [PMID: 20187168]
  • Hiromoto Yamakawa, Makoto Hakata. Atlas of rice grain filling-related metabolism under high temperature: joint analysis of metabolome and transcriptome demonstrated inhibition of starch accumulation and induction of amino acid accumulation. Plant & cell physiology. 2010 May; 51(5):795-809. doi: 10.1093/pcp/pcq034. [PMID: 20304786]
  • Ralph Kissen, Per Winge, Diem Hong Thi Tran, Tommy S Jørstad, Trond R Størseth, Tone Christensen, Atle M Bones. Transcriptional profiling of an Fd-GOGAT1/GLU1 mutant in Arabidopsis thaliana reveals a multiple stress response and extensive reprogramming of the transcriptome. BMC genomics. 2010 Mar; 11(?):190. doi: 10.1186/1471-2164-11-190. [PMID: 20307264]
  • Bianca T van Maldegem, Marinus Duran, Ronald J A Wanders, Hans R Waterham, Frits A Wijburg. Flavin adenine dinucleotide status and the effects of high-dose riboflavin treatment in short-chain acyl-CoA dehydrogenase deficiency. Pediatric research. 2010 Mar; 67(3):304-8. doi: 10.1203/pdr.0b013e3181cbd57b. [PMID: 19952864]
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  • Bianca T van Maldegem, Marinus Duran, Ronald J A Wanders, Hans R Waterham, Tom J de Koning, Estela Rubio, Frits A Wijburg. Fasting and fat-loading tests provide pathophysiological insight into short-chain acyl-coenzyme a dehydrogenase deficiency. The Journal of pediatrics. 2010 Jan; 156(1):121-7. doi: 10.1016/j.jpeds.2009.07.008. [PMID: 19800078]
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  • Eleanor W W Leung, Luke W Guddat. Conformational changes in a plant ketol-acid reductoisomerase upon Mg(2+) and NADPH binding as revealed by two crystal structures. Journal of molecular biology. 2009 May; 389(1):167-82. doi: 10.1016/j.jmb.2009.04.012. [PMID: 19362563]
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  • Patrícia Fernanda Schuck, Gustavo da Costa Ferreira, Carolina Maso Viegas, Anelise Miotti Tonin, Estela Natacha Brandt Busanello, Leticia Ferreira Pettenuzzo, Carlos Alexandre Netto, Moacir Wajner. Chronic early postnatal administration of ethylmalonic acid to rats causes behavioral deficit. Behavioural brain research. 2009 Feb; 197(2):364-70. doi: 10.1016/j.bbr.2008.09.031. [PMID: 18950661]
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