Methylglutaric acid (BioDeep_00000017772)

 

Secondary id: BioDeep_00000399991, BioDeep_00000595870

human metabolite PANOMIX_OTCML-2023 Endogenous blood metabolite BioNovoGene_Lab2019 Volatile Flavor Compounds natural product


代谢物信息卡片


3-Methylpentanedioic acid

化学式: C6H10O4 (146.057906)
中文名称: 3-甲基戊二酸, 甲基戊二酸
谱图信息: 最多检出来源 Homo sapiens(feces) 2.66%

Reviewed

Last reviewed on 2024-07-29.

Cite this Page

Methylglutaric acid. BioDeep Database v3. PANOMIX ltd, a top metabolomics service provider from China. https://query.biodeep.cn/s/methylglutaric_acid (retrieved 2024-11-22) (BioDeep RN: BioDeep_00000017772). 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)CC(=O)O
InChI: InChI=1S/C6H10O4/c1-4(2-5(7)8)3-6(9)10/h4H,2-3H2,1H3,(H,7,8)(H,9,10)

描述信息

Methylglutaric acid is a leucine metabolite. A large amount of methylglutaric acid is identified in urine of patients with deficiency of 3-methylglutaconyl coenzyme A hydratase (PMID 6181239). Methylglutaric acid is also found to be associated with 3-hydroxy-3-methylglutaryl-CoA lyase deficiency, another inborn error of metabolism.
Methylglutaric acid is a leucine metabolite. A large amount of methylglutaric acid is identified in urine of patients with deficiency of 3-methylglutaconyl coenzyme A hydratase (PMID 6181239). [HMDB]
3-Methylglutaric acid, a leucine metabolite, is a conspicuous C6 dicarboxylic organic acid classically associated with two distinct leucine pathway enzyme deficiencies, 3-hydroxy-3-methylglutaryl CoA lyase (HMGCL) and 3-methylglutaconyl CoA hydratase (AUH)[1][2].

同义名列表

18 个代谢物同义名

3-Methylpentanedioic acid; beta-Methyl-glutaric acid; beta-Methylglutaric acid; b-Methyl-glutaric acid; 3-Methyl-glutaric acid; Β-methylglutaric acid; 3-Methylpentanedioate; 3-METHYLGLUTARIC ACID; b-Methylglutaric acid; beta-Methylglutarate; Methylglutaric acid; 3-Methyl-glutarate; Β-methylglutarate; b-Methylglutarate; 3-Methylglutarate; Methylglutarate; Methylglutaric acid; 3-Methylglutaric acid



数据库引用编号

12 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)

9 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

  • Brian J Koos, Jeffrey A Gornbein. Early pregnancy metabolites predict gestational diabetes mellitus: implications for fetal programming. American journal of obstetrics and gynecology. 2021 02; 224(2):215.e1-215.e7. doi: 10.1016/j.ajog.2020.07.050. [PMID: 32739399]
  • Nevton Teixeira da Rosa-Junior, Belisa Parmeggiani, Nícolas Manzke Glänzel, Leonardo de Moura Alvorcem, Marina Rocha Frusciante, Carlos Severo Dutra Filho, Moacir Wajner, Guilhian Leipnitz. In vivo evidence that bezafibrate prevents oxidative stress and mitochondrial dysfunction caused by 3-methylglutaric acid in rat liver. Biochimie. 2020 Apr; 171-172(?):187-196. doi: 10.1016/j.biochi.2020.03.007. [PMID: 32169667]
  • Dylan E Jones, Leanne Perez, Robert O Ryan. 3-Methylglutaric acid in energy metabolism. Clinica chimica acta; international journal of clinical chemistry. 2020 Mar; 502(?):233-239. doi: 10.1016/j.cca.2019.11.006. [PMID: 31730811]
  • Camila Aguilar Delgado, Gilian Batista Balbueno Guerreiro, Carlos Eduardo Diaz Jacques, Daniella de Moura Coelho, Angela Sitta, Vanusa Manfredini, Moacir Wajner, Carmen Regla Vargas. Prevention by L-carnitine of DNA damage induced by 3-hydroxy-3-methylglutaric and 3-methylglutaric acids and experimental evidence of lipid and DNA damage in patients with 3-hydroxy-3-methylglutaric aciduria. Archives of biochemistry and biophysics. 2019 06; 668(?):16-22. doi: 10.1016/j.abb.2019.04.008. [PMID: 31047871]
  • Nevton Teixeira da Rosa-Junior, Belisa Parmeggiani, Mateus Struecker da Rosa, Nícolas Manzke Glänzel, Leonardo de Moura Alvorcem, Moacir Wajner, Guilhian Leipnitz. Bezafibrate In Vivo Administration Prevents 3-Methylglutaric Acid-Induced Impairment of Redox Status, Mitochondrial Biogenesis, and Neural Injury in Brain of Developing Rats. Neurotoxicity research. 2019 May; 35(4):809-822. doi: 10.1007/s12640-019-00019-9. [PMID: 30850947]
  • Dominique Roland, Patrice Jissendi-Tchofo, Gilbert Briand, Joseph Vamecq, Monique Fontaine, Vincent Ultré, Cécile Acquaviva-Bourdain, Karine Mention, Dries Dobbelaere. Coupled brain and urine spectroscopy - in vivo metabolomic characterization of HMG-CoA lyase deficiency in 5 patients. Molecular genetics and metabolism. 2017 06; 121(2):111-118. doi: 10.1016/j.ymgme.2017.03.006. [PMID: 28396157]
  • Ana Laura Colín-González, Ariana Lizbeth Paz-Loyola, María Eduarda de Lima, Sonia Galván-Arzate, Bianca Seminotti, César Augusto João Ribeiro, Guilhian Leipnitz, Diogo Onofre Souza, Moacir Wajner, Abel Santamaría. Experimental Evidence that 3-Methylglutaric Acid Disturbs Mitochondrial Function and Induced Oxidative Stress in Rat Brain Synaptosomes: New Converging Mechanisms. Neurochemical research. 2016 Oct; 41(10):2619-2626. doi: 10.1007/s11064-016-1973-2. [PMID: 27278758]
  • Mateus Struecker da Rosa, Bianca Seminotti, César Augusto João Ribeiro, Belisa Parmeggiani, Mateus Grings, Moacir Wajner, Guilhian Leipnitz. 3-Hydroxy-3-methylglutaric and 3-methylglutaric acids impair redox status and energy production and transfer in rat heart: relevance for the pathophysiology of cardiac dysfunction in 3-hydroxy-3-methylglutaryl-coenzyme A lyase deficiency. Free radical research. 2016 Sep; 50(9):997-1010. doi: 10.1080/10715762.2016.1214952. [PMID: 27430492]
  • S Sofer, A Schweiger, L Blumkin, G Yahalom, Y Anikster, D Lev, B Ben-Zeev, T Lerman-Sagie, S Hassin-Baer. The neuropsychological profile of patients with 3-methylglutaconic aciduria type III, Costeff syndrome. American journal of medical genetics. Part B, Neuropsychiatric genetics : the official publication of the International Society of Psychiatric Genetics. 2015 Apr; 168B(3):197-203. doi: 10.1002/ajmg.b.32296. [PMID: 25657044]
  • Carolina Gonçalves Fernandes, Mateus Struecker da Rosa, Bianca Seminotti, Paula Pierozan, Rafael Wolter Martell, Valeska Lizzi Lagranha, Estela Natacha Brandt Busanello, Guilhian Leipnitz, Moacir Wajner. In vivo experimental evidence that the major metabolites accumulating in 3-hydroxy-3-methylglutaryl-CoA lyase deficiency induce oxidative stress in striatum of developing rats: a potential pathophysiological mechanism of striatal damage in this disorder. Molecular genetics and metabolism. 2013 Jun; 109(2):144-53. doi: 10.1016/j.ymgme.2013.03.017. [PMID: 23611578]
  • Francesca Santarelli, Michela Cassanello, Ausilia Enea, Francesca Poma, Valentina D'Onofrio, Giovanna Guala, Giangiacomo Garrone, Paola Puccinelli, Ubaldo Caruso, Francesco Porta, Marco Spada. A neonatal case of 3-hydroxy-3-methylglutaric-coenzyme A lyase deficiency. Italian journal of pediatrics. 2013 May; 39(?):33. doi: 10.1186/1824-7288-39-33. [PMID: 23705938]
  • Saskia B Wortmann, Leo A Kluijtmans, Udo F H Engelke, Ron A Wevers, Eva Morava. The 3-methylglutaconic acidurias: what's new?. Journal of inherited metabolic disease. 2012 Jan; 35(1):13-22. doi: 10.1007/s10545-010-9210-7. [PMID: 20882351]
  • Marjan Huizing, Heidi Dorward, Lien Ly, Enriko Klootwijk, Robert Kleta, Flemming Skovby, Wuhong Pei, Benjamin Feldman, William A Gahl, Yair Anikster. OPA3, mutated in 3-methylglutaconic aciduria type III, encodes two transcripts targeted primarily to mitochondria. Molecular genetics and metabolism. 2010 Jun; 100(2):149-54. doi: 10.1016/j.ymgme.2010.03.005. [PMID: 20350831]
  • Guilhian Leipnitz, Bianca Seminotti, Carolina G Fernandes, Alexandre U Amaral, Ana Paula Beskow, Lucila de B da Silva, Angela Zanatta, César A J Ribeiro, Carmen R Vargas, Moacir Wajner. Striatum is more vulnerable to oxidative damage induced by the metabolites accumulating in 3-hydroxy-3-methylglutaryl-CoA lyase deficiency as compared to liver. International journal of developmental neuroscience : the official journal of the International Society for Developmental Neuroscience. 2009 Jun; 27(4):351-6. doi: 10.1016/j.ijdevneu.2009.03.001. [PMID: 19460629]
  • G Ho, J H Walter, J Christodoulou. Costeff optic atrophy syndrome: new clinical case and novel molecular findings. Journal of inherited metabolic disease. 2008 Dec; 31 Suppl 2(?):S419-23. doi: 10.1007/s10545-008-0981-z. [PMID: 18985435]
  • Guilhian Leipnitz, Bianca Seminotti, Alexandre U Amaral, Giorgia de Bortoli, Alexandre Solano, Patrícia F Schuck, Angela T S Wyse, Clóvis M D Wannmacher, Alexandra Latini, Moacir Wajner. Induction of oxidative stress by the metabolites accumulating in 3-methylglutaconic aciduria in cerebral cortex of young rats. Life sciences. 2008 Mar; 82(11-12):652-62. doi: 10.1016/j.lfs.2007.12.024. [PMID: 18261750]
  • Nuria Muñoz Jareño, Daniel Martín Fernández-Mayoralas, Celia Pérez-Cerdá Silvestre, Begoña Merinero Cortés, Magdalena Ugarte Pérez, Jaime Campos-Castelló. 3-methylglutaconic aciduria type 4 manifesting as Leigh syndrome in 2 siblings. Journal of child neurology. 2007 Feb; 22(2):218-21. doi: 10.1177/0883073807300300. [PMID: 17621487]
  • Udo F H Engelke, Berry Kremer, Leo A J Kluijtmans, Marinette van der Graaf, Eva Morava, Ference J Loupatty, Ronald J A Wanders, Detlef Moskau, Sandra Loss, Erik van den Bergh, Ron A Wevers. NMR spectroscopic studies on the late onset form of 3-methylglutaconic aciduria type I and other defects in leucine metabolism. NMR in biomedicine. 2006 Apr; 19(2):271-8. doi: 10.1002/nbm.1018. [PMID: 16541463]
  • L K Law, N L S Tang, J Hui, C W K Lam, T F Fok. 3-methyglutaconic aciduria in a Chinese patient with glycogen storage disease Ib. Journal of inherited metabolic disease. 2003; 26(7):705-9. doi: 10.1023/b:boli.0000005603.04633.21. [PMID: 14707520]
  • Robert Kleta, Flemming Skovby, Ernst Christensen, Thomas Rosenberg, William A Gahl, Yair Anikster. 3-Methylglutaconic aciduria type III in a non-Iraqi-Jewish kindred: clinical and molecular findings. Molecular genetics and metabolism. 2002 Jul; 76(3):201-6. doi: 10.1016/s1096-7192(02)00047-1. [PMID: 12126933]
  • Y Anikster, R Kleta, A Shaag, W A Gahl, O Elpeleg. Type III 3-methylglutaconic aciduria (optic atrophy plus syndrome, or Costeff optic atrophy syndrome): identification of the OPA3 gene and its founder mutation in Iraqi Jews. American journal of human genetics. 2001 Dec; 69(6):1218-24. doi: 10.1086/324651. [PMID: 11668429]
  • S Funghini, E Pasquini, M Cappellini, M A Donati, A Morrone, C Fonda, E Zammarchi. 3-Hydroxy-3-methylglutaric aciduria in an Italian patient is caused by a new nonsense mutation in the HMGCL gene. Molecular genetics and metabolism. 2001 Jul; 73(3):268-75. doi: 10.1006/mgme.2001.3191. [PMID: 11461194]
  • F Scaglia, V R Sutton, O A Bodamer, H Vogel, S K Shapira, R K Naviaux, G D Vladutiu. Mitochondrial DNA depletion associated with partial complex II and IV deficiencies and 3-methylglutaconic aciduria. Journal of child neurology. 2001 Feb; 16(2):136-8. doi: 10.1177/088307380101600214. [PMID: 11292221]
  • R Ensenauer, C B Müller, K O Schwab, K M Gibson, M Brandis, W Lehnert. 3-Methylglutaconyl-CoA hydratase deficiency: a new patient with speech retardation as the leading sign. Journal of inherited metabolic disease. 2000 Jun; 23(4):341-4. doi: 10.1023/a:1005670911799. [PMID: 10896289]
  • M Michelson, S Harel, A Gutman, T Lerman-Sagie. Urinary organic acid screening in children with developmental language delay. Journal of inherited metabolic disease. 1999 Oct; 22(7):815-20. doi: 10.1023/a:1005558108125. [PMID: 10518282]
  • R Walsh, H Conway, G Roche, P D Mayne. What is the origin of 3-methylglutaconic acid?. Journal of inherited metabolic disease. 1999 May; 22(3):251-5. doi: 10.1023/a:1005573819137. [PMID: 10384380]
  • T Lerman-Sagie. Behr syndrome. Pediatric neurology. 1995 Jan; 12(1):90. doi: 10.1016/0887-8994(95)95022-u. [PMID: 7538304]
  • O N Elpeleg, H Costeff, A Joseph, Y Shental, R Weitz, K M Gibson. 3-Methylglutaconic aciduria in the Iraqi-Jewish 'optic atrophy plus' (Costeff) syndrome. Developmental medicine and child neurology. 1994 Feb; 36(2):167-72. doi: 10.1111/j.1469-8749.1994.tb11825.x. [PMID: 7510656]
  • H Ibel, W Endres, H B Hadorn, T Deufel, I Paetzke, M Duran, N G Kennaway, K M Gibson. Multiple respiratory chain abnormalities associated with hypertrophic cardiomyopathy and 3-methylglutaconic aciduria. European journal of pediatrics. 1993 Aug; 152(8):665-70. doi: 10.1007/bf01955244. [PMID: 7691603]
  • R N Sheffer, J Zlotogora, O N Elpeleg, J Raz, D Ben-Ezra. Behr's syndrome and 3-methylglutaconic aciduria. American journal of ophthalmology. 1992 Oct; 114(4):494-7. doi: 10.1016/s0002-9394(14)71864-1. [PMID: 1384336]
  • T Kuhara, I Matsumoto, K Saiki, H Takabayashi, S Kuwabara. 3-Methylglutaconic aciduria in two adults. Clinica chimica acta; international journal of clinical chemistry. 1992 Apr; 207(1-2):151-3. doi: 10.1016/0009-8981(92)90159-n. [PMID: 1375542]
  • D Chitayat, J Chemke, K M Gibson, O A Mamer, J B Kronick, J J McGill, B Rosenblatt, L Sweetman, C R Scriver. 3-Methylglutaconic aciduria: a marker for as yet unspecified disorders and the relevance of prenatal diagnosis in a 'new' type ('type 4'). Journal of inherited metabolic disease. 1992; 15(2):204-12. doi: 10.1007/bf01799632. [PMID: 1382150]
  • K M Gibson, C F Lee, R S Wappner. 3-Methylglutaconyl-coenzyme-A hydratase deficiency: a new case. Journal of inherited metabolic disease. 1992; 15(3):363-6. doi: 10.1007/bf02435977. [PMID: 1383605]
  • R I Kelley, J P Cheatham, B J Clark, M A Nigro, B R Powell, G W Sherwood, J T Sladky, W P Swisher. X-linked dilated cardiomyopathy with neutropenia, growth retardation, and 3-methylglutaconic aciduria. The Journal of pediatrics. 1991 Nov; 119(5):738-47. doi: 10.1016/s0022-3476(05)80289-6. [PMID: 1719174]
  • K M Gibson, W G Sherwood, G F Hoffman, D A Stumpf, I Dianzani, R B Schutgens, P G Barth, U Weismann, C Bachmann, P Schrynemackers-Pitance. Phenotypic heterogeneity in the syndromes of 3-methylglutaconic aciduria. The Journal of pediatrics. 1991 Jun; 118(6):885-90. doi: 10.1016/s0022-3476(05)82199-7. [PMID: 1710267]
  • E A Haan, R D Scholem, J J Pitt, J E Wraith, G K Brown. Episodes of severe metabolic acidosis in a patient with 3-methylglutaconic aciduria. European journal of pediatrics. 1987 Sep; 146(5):484-8. doi: 10.1007/bf00441599. [PMID: 2445577]
  • W Lehnert, J Scharf, U Wendel. 3-Methylglutaconic and 3-methylglutaric aciduria in a patient with suspected 3-methylglutaconyl-CoA hydratase deficiency. European journal of pediatrics. 1985 Mar; 143(4):301-3. doi: 10.1007/bf00442306. [PMID: 2580710]
  • J Hammond, B Wilcken. 3-hydroxy-3-methylglutaric, 3-methylglutaconic and 3-methylglutaric acids can be non-specific indicators of metabolic disease. Journal of inherited metabolic disease. 1984; 7 Suppl 2(?):117-8. doi: 10.1007/978-94-009-5612-4_33. [PMID: 6207381]
  • B Hagberg, O Hjalmarson, S Lindstedt, L Ransnäs, G Steen. 3-Methylglutaconic aciduria in two infants. Clinica chimica acta; international journal of clinical chemistry. 1983 Oct; 134(1-2):59-67. doi: 10.1016/0009-8981(83)90184-5. [PMID: 6197209]
  • M Duran, F A Beemer, A S Tibosch, L Bruinvis, D Ketting, S K Wadman. Inherited 3-methylglutaconic aciduria in two brothers--another defect of leucine metabolism. The Journal of pediatrics. 1982 Oct; 101(4):551-4. doi: 10.1016/s0022-3476(82)80698-7. [PMID: 6181239]
  • E J Norman, M D Denton, H K Berry. Gas-chromatographic/mass spectrometric detection of 3-hydroxy-3-methylglutaryl-CoA lyase deficiency in double first cousins. Clinical chemistry. 1982 Jan; 28(1):137-40. doi: . [PMID: 7055897]