Butyrylcarnitine (BioDeep_00000003496)

 

Secondary id: BioDeep_00001869101, BioDeep_00001871813

human metabolite PANOMIX_OTCML-2023 Endogenous blood metabolite BioNovoGene_Lab2019


代谢物信息卡片


(3R)-3-(Butyryloxy)-4-(trimethylammonio)butanoic acid

化学式: C11H21NO4 (231.1470506)
中文名称: 丁酰肉碱
谱图信息: 最多检出来源 Homo sapiens(blood) 0.18%

Reviewed

Last reviewed on 2024-09-13.

Cite this Page

Butyrylcarnitine. BioDeep Database v3. PANOMIX ltd, a top metabolomics service provider from China. https://query.biodeep.cn/s/butyrylcarnitine (retrieved 2024-11-22) (BioDeep RN: BioDeep_00000003496). Licensed under the Attribution-Noncommercial 4.0 International License (CC BY-NC 4.0).

分子结构信息

SMILES: CCCC(=O)OC(CC(=O)[O-])C[N+](C)(C)C
InChI: InChI=1S/C11H21NO4/c1-5-6-11(15)16-9(7-10(13)14)8-12(2,3)4/h9H,5-8H2,1-4H3

描述信息

Butyrylcarnitine, also known as (3R)-3-(butyryloxy)-4-(trimethylammonio)butanoate or L-carnitine butyryl ester, is classified as a member of the acylcarnitines. Acylcarnitines are organic compounds containing a fatty acid with the carboxylic acid attached to carnitine through an ester bond. Butyrylcarnitine is considered to be practically insoluble (in water) and acidic. Butyrylcarnitine is elevated in patients with short-chain acyl-CoA dehydrogenase (SCAD) deficiency, in infants with acute acidosis and generalized muscle weakness, and in middle-aged patients with chronic myopathy localized in muscle (OMIM: 201470).
Butyrylcarnitine is elevated in patients with acyl-coa dehydrogenase, short-chain (SCAD) deficiencyin; in infants with acute acidosis and generalized muscle weakness; and in middle-aged patients with chronic myopathy localized in muscle. (OMIM 201470) [HMDB]
Butyrylcarnitine is a metabolite in plasma, acts as a biomarker to improve the diagnosis and prognosis of heart failure, and is indicative of anomalous lipid and energy metabolism.

同义名列表

11 个代谢物同义名

(3R)-3-(Butyryloxy)-4-(trimethylammonio)butanoic acid; (3R)-3-(butanoyloxy)-4-(trimethylazaniumyl)butanoate; (3R)-3-(Butyryloxy)-4-(trimethylammonio)butanoate; L-Carnitine butyryl ester; O-Butanoyl-(R)-carnitine; N-Butyryl-L-(-)-carnitin; O-butanoyl-carnitine; O-Butanoylcarnitine; Butyryl-L-carnitine; Butyrylcarnitine; Butylcarnitine



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(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)

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

  • Salem Werdyani, Ming Liu, Hongwei Zhang, Guang Sun, Andrew Furey, Edward W Randell, Proton Rahman, Guangju Zhai. Endotypes of primary osteoarthritis identified by plasma metabolomics analysis. Rheumatology (Oxford, England). 2021 06; 60(6):2735-2744. doi: 10.1093/rheumatology/keaa693. [PMID: 33159799]
  • Yefei Huang, Min Xiao, Jiayong Ou, Qing Lv, Qiujing Wei, Zena Chen, Jialing Wu, Liudan Tu, Yutong Jiang, Xi Zhang, Jun Qi, Minli Qiu, Shuangyan Cao, Jieruo Gu. Identification of the urine and serum metabolomics signature of gout. Rheumatology (Oxford, England). 2020 10; 59(10):2960-2969. doi: 10.1093/rheumatology/keaa018. [PMID: 32134107]
  • Ryusuke Hatae, Kenji Chamoto, Young Hak Kim, Kazuhiro Sonomura, Kei Taneishi, Shuji Kawaguchi, Hironori Yoshida, Hiroaki Ozasa, Yuichi Sakamori, Maryam Akrami, Sidonia Fagarasan, Izuru Masuda, Yasushi Okuno, Fumihiko Matsuda, Toyohiro Hirai, Tasuku Honjo. Combination of host immune metabolic biomarkers for the PD-1 blockade cancer immunotherapy. JCI insight. 2020 01; 5(2):. doi: 10.1172/jci.insight.133501. [PMID: 31855576]
  • 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]
  • Mark Haid, Caroline Muschet, Simone Wahl, Werner Römisch-Margl, Cornelia Prehn, Gabriele Möller, Jerzy Adamski. Long-Term Stability of Human Plasma Metabolites during Storage at -80 °C. Journal of proteome research. 2018 01; 17(1):203-211. doi: 10.1021/acs.jproteome.7b00518. [PMID: 29064256]
  • T Pallister, M A Jackson, T C Martin, C A Glastonbury, A Jennings, M Beaumont, R P Mohney, K S Small, A MacGregor, C J Steves, A Cassidy, T D Spector, C Menni, A M Valdes. Untangling the relationship between diet and visceral fat mass through blood metabolomics and gut microbiome profiling. International journal of obesity (2005). 2017 07; 41(7):1106-1113. doi: 10.1038/ijo.2017.70. [PMID: 28293020]
  • Mei-Ling Cheng, Chao-Hung Wang, Ming-Shi Shiao, Min-Hui Liu, Yu-Yen Huang, Cheng-Yu Huang, Chun-Tai Mao, Jui-Fen Lin, Hung-Yao Ho, Ning-I Yang. Metabolic disturbances identified in plasma are associated with outcomes in patients with heart failure: diagnostic and prognostic value of metabolomics. Journal of the American College of Cardiology. 2015 Apr; 65(15):1509-20. doi: 10.1016/j.jacc.2015.02.018. [PMID: 25881932]
  • 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]
  • Sabrina Forni, Xiaowei Fu, Susan E Palmer, Lawrence Sweetman. Rapid determination of C4-acylcarnitine and C5-acylcarnitine isomers in plasma and dried blood spots by UPLC-MS/MS as a second tier test following flow-injection MS/MS acylcarnitine profile analysis. Molecular genetics and metabolism. 2010 Sep; 101(1):25-32. doi: 10.1016/j.ymgme.2010.05.012. [PMID: 20591710]
  • 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]
  • Stuart G Beattie, Eric Goetzman, Thomas Conlon, Sean Germain, Glenn Walter, Martha Campbell-Thompson, Dietrich Matern, Jerry Vockley, Terence R Flotte. Biochemical correction of short-chain acyl-coenzyme A dehydrogenase deficiency after portal vein injection of rAAV8-SCAD. Human gene therapy. 2008 Jun; 19(6):579-88. doi: 10.1089/hum.2007.168. [PMID: 18500942]
  • B Merinero, C Pérez-Cerdá, P Ruiz Sala, I Ferrer, M J García, M Martínez Pardo, A Belanger-Quintana, J L de la Mota, E Martin-Hernández, C Vianey-Saban, C Bischoff, N Gregersen, M Ugarte. Persistent increase of plasma butyryl/isobutyrylcarnitine concentrations as marker of SCAD defect and ethylmalonic encephalopathy. Journal of inherited metabolic disease. 2006 Oct; 29(5):685. doi: 10.1007/s10545-006-0342-8. [PMID: 16906473]
  • Bianca T van Maldegem, Marinus Duran, Ronald J A Wanders, Klary E Niezen-Koning, Marije Hogeveen, Lodewijk Ijlst, Hans R Waterham, Frits A Wijburg. Clinical, biochemical, and genetic heterogeneity in short-chain acyl-coenzyme A dehydrogenase deficiency. JAMA. 2006 Aug; 296(8):943-52. doi: 10.1001/jama.296.8.943. [PMID: 16926354]
  • Alexandra Hack, Verena Busch, Bettina Pascher, Raymonde Busch, Iris Bieger, Klaus Gempel, Friedrich A M Baumeister. Monitoring of ketogenic diet for carnitine metabolites by subcutaneous microdialysis. Pediatric research. 2006 Jul; 60(1):93-6. doi: 10.1203/01.pdr.0000219479.95410.79. [PMID: 16690958]
  • Thomas J Conlon, Glenn Walter, Renius Owen, Travis Cossette, Kirsten Erger, Greg Gutierrez, Eric Goetzman, Dietrich Matern, Jerry Vockley, Terence R Flotte. Systemic correction of a fatty acid oxidation defect by intramuscular injection of a recombinant adeno-associated virus vector. Human gene therapy. 2006 Jan; 17(1):71-80. doi: 10.1089/hum.2006.17.71. [PMID: 16409126]
  • Neil Gordon. Acyl-CoA dehydrogenase deficiency: varieties with neurological involvement. Developmental medicine and child neurology. 2005 Mar; 47(3):207-10. doi: 10.1017/s0012162205000381. [PMID: 15739728]
  • Sarah P Young, Dietrich Matern, Niels Gregersen, Robert D Stevens, Deeksha Bali, Hui-Ming Liu, Dwight D Koeberl, David S Millington. A comparison of in vitro acylcarnitine profiling methods for the diagnosis of classical and variant short chain acyl-CoA dehydrogenase deficiency. Clinica chimica acta; international journal of clinical chemistry. 2003 Nov; 337(1-2):103-13. doi: 10.1016/j.cccn.2003.07.006. [PMID: 14568186]
  • Dwight D Koeberl, Sarah P Young, Niels S Gregersen, Jerry Vockley, Wendy E Smith, Daniel Kelly Benjamin, Yan An, Susan D Weavil, Shu H Chaing, Deeksha Bali, Marie T McDonald, Priya S Kishnani, Y-T Chen, David S Millington. Rare disorders of metabolism with elevated butyryl- and isobutyryl-carnitine detected by tandem mass spectrometry newborn screening. Pediatric research. 2003 Aug; 54(2):219-23. doi: 10.1203/01.pdr.0000074972.36356.89. [PMID: 12736383]
  • I Tamai, R Ohashi, J I Nezu, Y Sai, D Kobayashi, A Oku, M Shimane, A Tsuji. Molecular and functional characterization of organic cation/carnitine transporter family in mice. The Journal of biological chemistry. 2000 Dec; 275(51):40064-72. doi: 10.1074/jbc.m005340200. [PMID: 11010964]