Deoxycarnitine (BioDeep_00000018396)

 

Secondary id: BioDeep_00001871872

human metabolite Endogenous blood metabolite


代谢物信息卡片


4-(N-Trimethylamino)butyric acid

化学式: C7H15NO2 (145.110273)
中文名称: (3-羧丙基)三甲基氯化铵
谱图信息: 最多检出来源 Homo sapiens(blood) 0.01%

Reviewed

Last reviewed on 2024-09-13.

Cite this Page

Deoxycarnitine. BioDeep Database v3. PANOMIX ltd, a top metabolomics service provider from China. https://query.biodeep.cn/s/deoxycarnitine (retrieved 2024-11-08) (BioDeep RN: BioDeep_00000018396). Licensed under the Attribution-Noncommercial 4.0 International License (CC BY-NC 4.0).

分子结构信息

SMILES: C[N+](C)(C)CCCC(=O)[O-]
InChI: InChI=1S/C7H15NO2/c1-8(2,3)6-4-5-7(9)10/h4-6H2,1-3H3

描述信息

4-Trimethylammoniobutanoic acid, also known as gamma-butyrobetaine (GBB) or 3-dehydroxycarnitine, is a highly water-soluble derivative of gamma-aminobutyric acid (GABA). It is also a precursor of L-carnitine. It is a substrate of gamma butyrobetaine hydroxylase/dioxygenase (also known as BBOX) which catalyzes the formation of L-carnitine from gamma-butyrobetaine, the last step in the L-carnitine biosynthesis pathway. Carnitine is essential for the transport of activated fatty acids across the mitochondrial membrane during mitochondrial beta-oxidation. Numerous disorders have been described that lead to disturbances in energy production and in intermediary metabolism which are characterized by the production and excretion of unusual acylcarnitines. A mutation in the gene coding for carnitine-acylcarnitine translocase, or the OCTN2 transporter aetiologically, causes a carnitine deficiency that results in poor intestinal absorption of dietary L-carnitine, impaired reabsorption by the kidney, and increased urinary loss. Determination of the qualitative pattern of acylcarnitines can be of diagnostic and therapeutic importance. The betaine structure of carnitine requires special analytical procedures for recording. The ionic nature of L-carnitine causes a high water solubility which decreases with increasing chain length of the ester group in the acylcarnitines. Therefore, the distribution of L-carnitine and acylcarnitines in various organs is defined by their function and their physicochemical properties as well. High-performance liquid chromatography (HPLC) permits screening for free and total carnitine, as well as complete quantitative acylcarnitine determination, including the long-chain acylcarnitine profile (PMID: 17508264, Monatshefte fuer Chemie (2005), 136(8), 1279-1291., Int J Mass Spectrom. 1999;188:39-52.).
3-Dehydroxycarnitine is an acylcarnitine. Numerous disorders have been described that lead to disturbances in energy production and in intermediary metabolism in the organism which are characterized by the production and excretion of unusual acylcarnitines. A mutation in the gene coding for carnitine-acylcarnitine translocase or the OCTN2 transporter aetiologically causes a carnitine deficiency that results in poor intestinal absorption of dietary L-carnitine, its impaired reabsorption by the kidney and, consequently, in increased urinary loss of L-carnitine. Determination of the qualitative pattern of acylcarnitines can be of diagnostic and therapeutic importance. The betaine structure of carnitine requires special analytical procedures for recording. The ionic nature of L-carnitine causes a high water solubility which decreases with increasing chain length of the ester group in the acylcarnitines. Therefore, the distribution of L-carnitine and acylcarnitines in various organs is defined by their function and their physico-chemical properties as well. High performance liquid chromatography (HPLC) permits screening for free and total carnitine, as well as complete quantitative acylcarnitine determination, including the long-chain acylcarnitine profile. (PMID: 17508264, Monatshefte fuer Chemie (2005), 136(8), 1279-1291., Int J Mass Spectrom. 1999;188:39-52.) [HMDB]
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同义名列表

22 个代谢物同义名

4-(N-Trimethylamino)butyric acid; 4-(Trimethylamino)butanoic acid; 4-(trimethylazaniumyl)butanoate; 4-Trimethylammoniobutanoic acid; 4-N-Trimethylammonium butyrate; 4-(Trimethylammonio)butanoate; 4-(N-Trimethylamino)butyrate; 4-Trimethylammoniobutanoate; 4-(Trimethylamino)butanoate; 4-Trimethylaminobutyrate; 3-Dehydroxycarnitine; gamma-Butyrobetaine; gamma-Butyrobetain; Γ-butyrobetaine; Deoxy-carnitine; 4-Butyrobetaine; g-Butyrobetaine; g-Butyrobetain; Γ-butyrobetain; Deoxycarnitine; Butyrobetaine; Actinine



数据库引用编号

11 个数据库交叉引用编号

分类词条

相关代谢途径

Reactome(2)

BioCyc(2)

PlantCyc(0)

代谢反应

69 个相关的代谢反应过程信息。

Reactome(48)

BioCyc(14)

WikiPathways(1)

Plant Reactome(0)

INOH(0)

PlantCyc(3)

COVID-19 Disease Map(1)

PathBank(2)

  • L-Carnitine Degradation I: Adenosine triphosphate + Coenzyme A + L-Carnitine ⟶ Adenosine monophosphate + L-Carnitinyl-CoA + diphosphate
  • L-Carnitine Degradation I: Adenosine triphosphate + Coenzyme A + L-Carnitine ⟶ Adenosine monophosphate + L-Carnitinyl-CoA + diphosphate

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

  • Haoran Wei, Mingming Zhao, Junfang Wu, Chenze Li, Man Huang, Jianing Gao, Qi Zhang, Liang Ji, Yan Wang, Chunxia Zhao, Erdan Dong, Lemin Zheng, Dao Wen Wang. Association of Systemic Trimethyllysine With Heart Failure With Preserved Ejection Fraction and Cardiovascular Events. The Journal of clinical endocrinology and metabolism. 2022 11; 107(12):e4360-e4370. doi: 10.1210/clinem/dgac519. [PMID: 36062477]
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  • Florian Jacques, Yingjuan Zhao, Martina Kopečná, Radka Končitíková, David Kopečný, Sonia Rippa, Yolande Perrin. Roles for ALDH10 enzymes in γ-butyrobetaine synthesis, seed development, germination, and salt tolerance in Arabidopsis. Journal of experimental botany. 2020 12; 71(22):7088-7102. doi: 10.1093/jxb/eraa394. [PMID: 32845293]
  • Lu Chen, Yong Chen, Mingming Zhao, Lemin Zheng, Dongsheng Fan. Changes in the concentrations of trimethylamine N-oxide (TMAO) and its precursors in patients with amyotrophic lateral sclerosis. Scientific reports. 2020 09; 10(1):15198. doi: 10.1038/s41598-020-72184-3. [PMID: 32938991]
  • Nesrin Damla Eyupoglu, Ezgi Caliskan Guzelce, Aylin Acikgoz, Esra Uyanik, Bodil Bjørndal, Rolf K Berge, Asbjørn Svardal, Bulent Okan Yildiz. Circulating gut microbiota metabolite trimethylamine N-oxide and oral contraceptive use in polycystic ovary syndrome. Clinical endocrinology. 2019 12; 91(6):810-815. doi: 10.1111/cen.14101. [PMID: 31556132]
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  • Pascal Bazire, Nadia Perchat, Ekaterina Darii, Christophe Lechaplais, Marcel Salanoubat, Alain Perret. Characterization of l-Carnitine Metabolism in Sinorhizobium meliloti. Journal of bacteriology. 2019 04; 201(7):. doi: 10.1128/jb.00772-18. [PMID: 30670548]
  • Robert A Koeth, Betzabe Rachel Lam-Galvez, Jennifer Kirsop, Zeneng Wang, Bruce S Levison, Xiaodong Gu, Matthew F Copeland, David Bartlett, David B Cody, Hong J Dai, Miranda K Culley, Xinmin S Li, Xiaoming Fu, Yuping Wu, Lin Li, Joseph A DiDonato, W H Wilson Tang, Jose Carlos Garcia-Garcia, Stanley L Hazen. l-Carnitine in omnivorous diets induces an atherogenic gut microbial pathway in humans. The Journal of clinical investigation. 2019 01; 129(1):373-387. doi: 10.1172/jci94601. [PMID: 30530985]
  • Aarti Gautam, Seid Muhie, Nabarun Chakraborty, Allison Hoke, Duncan Donohue, Stacy Ann Miller, Rasha Hammamieh, Marti Jett. Metabolomic analyses reveal lipid abnormalities and hepatic dysfunction in non-human primate model for Yersinia pestis. Metabolomics : Official journal of the Metabolomic Society. 2018 12; 15(1):2. doi: 10.1007/s11306-018-1457-2. [PMID: 30830480]
  • Dorottya Nagy-Szakal, Dinesh K Barupal, Bohyun Lee, Xiaoyu Che, Brent L Williams, Ellie J R Kahn, Joy E Ukaigwe, Lucinda Bateman, Nancy G Klimas, Anthony L Komaroff, Susan Levine, Jose G Montoya, Daniel L Peterson, Bruce Levin, Mady Hornig, Oliver Fiehn, W Ian Lipkin. Insights into myalgic encephalomyelitis/chronic fatigue syndrome phenotypes through comprehensive metabolomics. Scientific reports. 2018 07; 8(1):10056. doi: 10.1038/s41598-018-28477-9. [PMID: 29968805]
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  • Marius Trøseid, Johannes R Hov, Torunn Kristin Nestvold, Hanne Thoresen, Rolf K Berge, Asbjørn Svardal, Knut Tore Lappegård. Major Increase in Microbiota-Dependent Proatherogenic Metabolite TMAO One Year After Bariatric Surgery. Metabolic syndrome and related disorders. 2016 May; 14(4):197-201. doi: 10.1089/met.2015.0120. [PMID: 27081744]
  • Karolina Skagen, Marius Trøseid, Thor Ueland, Sverre Holm, Azhar Abbas, Ida Gregersen, Martin Kummen, Vigdis Bjerkeli, Frode Reier-Nilsen, David Russell, Asbjørn Svardal, Tom Hemming Karlsen, Pål Aukrust, Rolf K Berge, Johannes E R Hov, Bente Halvorsen, Mona Skjelland. The Carnitine-butyrobetaine-trimethylamine-N-oxide pathway and its association with cardiovascular mortality in patients with carotid atherosclerosis. Atherosclerosis. 2016 Apr; 247(?):64-9. doi: 10.1016/j.atherosclerosis.2016.01.033. [PMID: 26868510]
  • Paul E Minkler, Maria S K Stoll, Stephen T Ingalls, Janos Kerner, Charles L Hoppel. Quantitative acylcarnitine determination by UHPLC-MS/MS--Going beyond tandem MS acylcarnitine "profiles". Molecular genetics and metabolism. 2015 Dec; 116(4):231-41. doi: 10.1016/j.ymgme.2015.10.002. [PMID: 26458767]
  • Solveiga Grinberga, Maija Dambrova, Gustavs Latkovskis, Ieva Strele, Ilze Konrade, Dace Hartmane, Eduards Sevostjanovs, Edgars Liepinsh, Osvalds Pugovics. Determination of trimethylamine-N-oxide in combination with L-carnitine and γ-butyrobetaine in human plasma by UPLC/MS/MS. Biomedical chromatography : BMC. 2015 Nov; 29(11):1670-4. doi: 10.1002/bmc.3477. [PMID: 25873316]
  • Paul E Minkler, Maria S K Stoll, Stephen T Ingalls, Janos Kerner, Charles L Hoppel. Validated method for the quantification of free and total carnitine, butyrobetaine, and acylcarnitines in biological samples. Analytical chemistry. 2015 Sep; 87(17):8994-9001. doi: 10.1021/acs.analchem.5b02198. [PMID: 26270397]
  • Reinis Vilskersts, Janis Kuka, Edgars Liepinsh, Marina Makrecka-Kuka, Kristine Volska, Elina Makarova, Eduards Sevostjanovs, Helena Cirule, Solveiga Grinberga, Maija Dambrova. Methyl-γ-butyrobetaine decreases levels of acylcarnitines and attenuates the development of atherosclerosis. Vascular pharmacology. 2015 Sep; 72(?):101-7. doi: 10.1016/j.vph.2015.05.005. [PMID: 25989106]
  • Janis Kuka, Edgars Liepinsh, Marina Makrecka-Kuka, Janis Liepins, Helena Cirule, Daina Gustina, Einars Loza, Olga Zharkova-Malkova, Solveiga Grinberga, Osvalds Pugovics, Maija Dambrova. Suppression of intestinal microbiota-dependent production of pro-atherogenic trimethylamine N-oxide by shifting L-carnitine microbial degradation. Life sciences. 2014 Nov; 117(2):84-92. doi: 10.1016/j.lfs.2014.09.028. [PMID: 25301199]
  • Takafumi Saito, Masahiro Sugimoto, Kaori Igarashi, Kaori Saito, Li Shao, Tomohiro Katsumi, Kyoko Tomita, Chikako Sato, Kazuo Okumoto, Yuko Nishise, Hisayoshi Watanabe, Masaru Tomita, Yoshiyuki Ueno, Tomoyoshi Soga. Dynamics of serum metabolites in patients with chronic hepatitis C receiving pegylated interferon plus ribavirin: a metabolomics analysis. Metabolism: clinical and experimental. 2013 Nov; 62(11):1577-86. doi: 10.1016/j.metabol.2013.07.002. [PMID: 23953890]
  • Jenna Pekkinen, Kaisa Olli, Anne Huotari, Kirsti Tiihonen, Pekka Keski-Rahkonen, Marko Lehtonen, Seppo Auriola, Marjukka Kolehmainen, Hannu Mykkänen, Kaisa Poutanen, Kati Hanhineva. Betaine supplementation causes increase in carnitine metabolites in the muscle and liver of mice fed a high-fat diet as studied by nontargeted LC-MS metabolomics approach. Molecular nutrition & food research. 2013 Nov; 57(11):1959-68. doi: 10.1002/mnfr.201300142. [PMID: 23868375]
  • Sonia Rippa, Yingjuan Zhao, Franck Merlier, Aurélie Charrier, Yolande Perrin. The carnitine biosynthetic pathway in Arabidopsis thaliana shares similar features with the pathway of mammals and fungi. Plant physiology and biochemistry : PPB. 2012 Nov; 60(?):109-14. doi: 10.1016/j.plaphy.2012.08.001. [PMID: 22922110]
  • Réjane Morand, Liliane Todesco, Massimiliano Donzelli, David Fischer-Barnicol, Peter J Mullen, Stephan Krähenbühl. Effect of short- and long-term treatment with valproate on carnitine homeostasis in humans. Therapeutic drug monitoring. 2012 Aug; 34(4):406-14. doi: 10.1097/ftd.0b013e3182608e2f. [PMID: 22743351]
  • Edgars Liepinsh, Ilze Konrade, Elina Skapare, Osvalds Pugovics, Solveiga Grinberga, Janis Kuka, Ivars Kalvinsh, Maija Dambrova. Mildronate treatment alters γ-butyrobetaine and l-carnitine concentrations in healthy volunteers. The Journal of pharmacy and pharmacology. 2011 Sep; 63(9):1195-201. doi: 10.1111/j.2042-7158.2011.01325.x. [PMID: 21827492]
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  • Sonja Primassin, Ute Spiekerkoetter. ESI-MS/MS measurement of free carnitine and its precursor γ-butyrobetaine in plasma and dried blood spots from patients with organic acidurias and fatty acid oxidation disorders. Molecular genetics and metabolism. 2010 Oct; 101(2-3):141-5. doi: 10.1016/j.ymgme.2010.06.012. [PMID: 20637671]
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