Isovalerylcarnitine (BioDeep_00000405335)

Main id: BioDeep_00000013676

 

PANOMIX_OTCML-2023


代谢物信息卡片


isovaleryl-L-carnitine

化学式: C12H23NO4 (245.16269979999998)
中文名称: 3-甲基丁酰卡尼汀
谱图信息: 最多检出来源 () 0%

分子结构信息

SMILES: CC(C)CC(=O)OC(CC(=O)[O-])C[N+](C)(C)C
InChI: InChI=1S/C12H23NO4/c1-9(2)6-12(16)17-10(7-11(14)15)8-13(3,4)5/h9-10H,6-8H2,1-5H3

描述信息

An O-isovalerylcarnitine that is the 3-methylbutanoyl (isovaleryl) derivative of L-carnitine.
Isovalerylcarnitine is a product of the catabolism of L-leucine. Isovalerylcarnitine is also a selective and reversible calpain activator that induces apoptosis[1][2][3].

同义名列表

2 个代谢物同义名

Isovalerylcarnitine; isovaleryl-L-carnitine



数据库引用编号

10 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)

3 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

  • María L Couce, Luís Aldamiz-Echevarría, María A Bueno, Patricia Barros, Amaya Belanger-Quintana, Javier Blasco, María-Teresa García-Silva, Ana M Márquez-Armenteros, Isidro Vitoria, Inmaculada Vives, Rosa Navarrete, Ana Fernández-Marmiesse, Belén Pérez, Celia Pérez-Cerdá. Genotype and phenotype characterization in a Spanish cohort with isovaleric acidemia. Journal of human genetics. 2017 Mar; 62(3):355-360. doi: 10.1038/jhg.2016.144. [PMID: 27904153]
  • Mariano Malaguarnera. Carnitine derivatives: clinical usefulness. Current opinion in gastroenterology. 2012 Mar; 28(2):166-76. doi: 10.1097/mog.0b013e3283505a3b. [PMID: 22333562]
  • C Danese, M Cirene, M Colotto, A Aratari, S Amato, S Di Bona, M Curione. Cardiac involvement in inflammatory bowel disease: role of acylcarnitine esters. La Clinica terapeutica. 2011; 162(4):e105-9. doi: . [PMID: 21912810]
  • Judit Bene, Katalin Komlósi, Viktória Havasi, Gábor Talián, Beáta Gasztonyi, Krisztina Horváth, Gyula Mózsik, Béla Hunyady, Béla Melegh, Mária Figler. Changes of plasma fasting carnitine ester profile in patients with ulcerative colitis. World journal of gastroenterology. 2006 Jan; 12(1):110-3. doi: 10.3748/wjg.v12.i1.110. [PMID: 16440427]
  • C R Roe, S D Cederbaum, D S Roe, R Mardach, A Galindo, L Sweetman. Isolated isobutyryl-CoA dehydrogenase deficiency: an unrecognized defect in human valine metabolism. Molecular genetics and metabolism. 1998 Dec; 65(4):264-71. doi: 10.1006/mgme.1998.2758. [PMID: 9889013]
  • J E Abdenur, N A Chamoles, A E Guinle, A B Schenone, A N Fuertes. Diagnosis of isovaleric acidaemia by tandem mass spectrometry: false positive result due to pivaloylcarnitine in a newborn screening programme. Journal of inherited metabolic disease. 1998 Aug; 21(6):624-30. doi: 10.1023/a:1005424331822. [PMID: 9762597]
  • M H Fries, P Rinaldo, E Schmidt-Sommerfeld, E Jurecki, S Packman. Isovaleric acidemia: response to a leucine load after three weeks of supplementation with glycine, L-carnitine, and combined glycine-carnitine therapy. The Journal of pediatrics. 1996 Sep; 129(3):449-52. doi: 10.1016/s0022-3476(96)70081-1. [PMID: 8804338]
  • F Salamino, F Di Lisa, A B Burlina, R Menabŏ, R Barbato, R De Tullio, N Siliprandi. Involvement of erythrocyte calpain in glycine- and carnitine-treated isovaleric acidemia. Pediatric research. 1994 Aug; 36(2):182-6. doi: 10.1203/00006450-199408000-00008. [PMID: 7970932]
  • G Miotto, R Venerando, K K Khurana, N Siliprandi, G E Mortimore. Control of hepatic proteolysis by leucine and isovaleryl-L-carnitine through a common locus. Evidence for a possible mechanism of recognition at the plasma membrane. The Journal of biological chemistry. 1992 Nov; 267(31):22066-72. doi: NULL. [PMID: 1429558]
  • T Sakuma, N Sugiyama, T Ichiki, M Kobayashi, Y Wada, D Nohara. Analysis of acylcarnitines in maternal urine for prenatal diagnosis of glutaric aciduria type 2. Prenatal diagnosis. 1991 Feb; 11(2):77-82. doi: 10.1002/pd.1970110203. [PMID: 2062823]
  • S Pontremoli, E Melloni, P L Viotti, M Michetti, F Di Lisa, N Siliprandi. Isovalerylcarnitine is a specific activator of the high calcium requiring calpain forms. Biochemical and biophysical research communications. 1990 Feb; 167(1):373-80. doi: 10.1016/0006-291x(90)91775-n. [PMID: 2310400]
  • P E Minkler, S T Ingalls, C L Hoppel. High-performance liquid chromatographic separation of acylcarnitines following derivatization with 4'-bromophenacyl trifluoromethanesulfonate. Analytical biochemistry. 1990 Feb; 185(1):29-35. doi: 10.1016/0003-2697(90)90250-d. [PMID: 2344045]
  • R A Chalmers, C R Roe, T E Stacey, C L Hoppel. Urinary excretion of l-carnitine and acylcarnitines by patients with disorders of organic acid metabolism: evidence for secondary insufficiency of l-carnitine. Pediatric research. 1984 Dec; 18(12):1325-8. doi: 10.1203/00006450-198412000-00021. [PMID: 6441143]
  • C R Roe, D S Millington, D A Maltby, S G Kahler, T P Bohan. L-carnitine therapy in isovaleric acidemia. The Journal of clinical investigation. 1984 Dec; 74(6):2290-5. doi: 10.1172/jci111657. [PMID: 6549017]
  • J Kerner, J A Froseth, E R Miller, L L Bieber. A study of the acylcarnitine content of sows' colostrum, milk and newborn piglet tissues: demonstration of high amounts of isovalerylcarnitine in colostrum and milk. The Journal of nutrition. 1984 May; 114(5):854-61. doi: 10.1093/jn/114.5.854. [PMID: 6726455]