Ubiquinone 6 (BioDeep_00000004131)

 

Secondary id: BioDeep_00000639384, BioDeep_00001891298

human metabolite Endogenous Volatile Flavor Compounds


代谢物信息卡片


2-[(2E,6E,10E,14E,18E)-3,7,11,15,19,23-hexamethyltetracosa-2,6,10,14,18,22-hexaen-1-yl]-5,6-dimethoxy-3-methylcyclohexa-2,5-diene-1,4-dione

化学式: C39H58O4 (590.4334868)
中文名称: 辅酶Q6
谱图信息: 最多检出来源 Homo sapiens(blood) 40.92%

分子结构信息

SMILES: CC1=C(C(=O)C(=C(C1=O)OC)OC)CC=C(C)CCC=C(C)CCC=C(C)CCC=C(C)CCC=C(C)CCC=C(C)C
InChI: InChI=1S/C39H58O4/c1-28(2)16-11-17-29(3)18-12-19-30(4)20-13-21-31(5)22-14-23-32(6)24-15-25-33(7)26-27-35-34(8)36(40)38(42-9)39(43-10)37(35)41/h16,18,20,22,24,26H,11-15,17,19,21,23,25,27H2,1-10H3/b29-18+,30-20+,31-22+,32-24+,33-26+

描述信息

Ubiquinone-6 is a member of the chemical class known as Polyprenylbenzoquinones. These are compounds containing a polyisoprene chain attached to a quinone at the second ring position. Ubiquione-6 has just 6 isoprene units. Normally in humans it has 10. Ubiquinone-6 is an intermediate in the synthesis of Ubiquionone 10. It is an endogenouse compound but it has also been isolated from foods containing bakers yeast. Ubiquionone 10 (CoQ10) is involved in cellular respiration. It is fat-soluble and is therefore mobile in cellular membranes; it plays a unique role in the electron transport chain (ETC). In the inner bacterial membrane, electrons from NADH and succinate pass through the ETC to the oxygen, which is then reduced to water. The transfer of electrons through ETC results in the pumping of H+ across the membrane creating a proton gradient across the membrane, which is used by ATP synthase (located on the membrane) to generate ATP.
Isolated from bakers yeast (Saccharomyces cerevisiae)

同义名列表

14 个代谢物同义名

2-[(2E,6E,10E,14E,18E)-3,7,11,15,19,23-hexamethyltetracosa-2,6,10,14,18,22-hexaen-1-yl]-5,6-dimethoxy-3-methylcyclohexa-2,5-diene-1,4-dione; 2-(3,7,11,15,19,23-hexamethyltetracosa-2,6,10,14,18,22-hexaen-1-yl)-5,6-dimethoxy-3-methylcyclohexa-2,5-diene-1,4-dione; 2,3-Dimethoxy-5-methyl-6-(farnesylfarnesyl)-1,4-benzoquinone; Ubiquinone(6); Ubiquinone Q6; Ubiquinone 30; ubiquinone-6; Ubiquinone 6; Coenzyme QQ6; Coenzyme Q6; Coenzyme-Q6; CoQ-6; CoQ6; Ubiquinone-6



数据库引用编号

16 个数据库交叉引用编号

分类词条

相关代谢途径

Reactome(0)

BioCyc(1)

PlantCyc(0)

代谢反应

19 个相关的代谢反应过程信息。

Reactome(0)

BioCyc(8)

WikiPathways(0)

Plant Reactome(0)

INOH(0)

PlantCyc(0)

COVID-19 Disease Map(0)

PathBank(11)

  • Methylglyoxal Degradation I: Glutathione + Pyruvaldehyde ⟶ S-Lactoylglutathione
  • Methylglyoxal Degradation I: Glutathione + Pyruvaldehyde ⟶ S-Lactoylglutathione
  • TCA cycle (ubiquinol-6): Citric acid ⟶ Water + cis-Aconitic acid
  • TCA Cycle (Ubiquinol-6): Water + cis-Aconitic acid ⟶ D-threo-Isocitric acid
  • Ubiquinone Biosynthesis: 3-Hexaprenyl-4,5-Dihydroxybenzoic acid + S-Adenosylmethionine ⟶ 3-Hexaprenyl-4-hydroxy-5-methoxybenzoic acid + S-Adenosylhomocysteine
  • Ubiquinone Biosynthesis: 3-Hexaprenyl-4,5-Dihydroxybenzoic acid + S-Adenosylmethionine ⟶ 3-Hexaprenyl-4-hydroxy-5-methoxybenzoic acid + S-Adenosylhomocysteine
  • Ubiquinone Biosynthesis: 3-Hexaprenyl-4,5-Dihydroxybenzoic acid + S-Adenosylmethionine ⟶ 3-Hexaprenyl-4-hydroxy-5-methoxybenzoic acid + S-Adenosylhomocysteine
  • Ubiquinone Biosynthesis: 3-Hexaprenyl-4,5-Dihydroxybenzoic acid + S-Adenosylmethionine ⟶ 3-Hexaprenyl-4-hydroxy-5-methoxybenzoic acid + S-Adenosylhomocysteine
  • Ubiquinone Biosynthesis: 3-Hexaprenyl-4,5-Dihydroxybenzoic acid + S-Adenosylmethionine ⟶ 3-Hexaprenyl-4-hydroxy-5-methoxybenzoic acid + S-Adenosylhomocysteine
  • Ubiquinone Biosynthesis: 3-Hexaprenyl-4,5-Dihydroxybenzoic acid + S-Adenosylmethionine ⟶ 3-Hexaprenyl-4-hydroxy-5-methoxybenzoic acid + S-Adenosylhomocysteine
  • Ubiquinone Biosynthesis: 3-Hexaprenyl-4,5-Dihydroxybenzoic acid + S-Adenosylmethionine ⟶ 3-Hexaprenyl-4-hydroxy-5-methoxybenzoic acid + S-Adenosylhomocysteine

PharmGKB(0)

2 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

  • Michelle C Bradley, Krista Yang, Lucía Fernández-Del-Río, Jennifer Ngo, Anita Ayer, Hui S Tsui, Noelle Alexa Novales, Roland Stocker, Orian S Shirihai, Mario H Barros, Catherine F Clarke. COQ11 deletion mitigates respiratory deficiency caused by mutations in the gene encoding the coenzyme Q chaperone protein Coq10. The Journal of biological chemistry. 2020 05; 295(18):6023-6042. doi: 10.1074/jbc.ra119.012420. [PMID: 32205446]
  • Zeynep Yuruk Yildirim, Guven Toksoy, Oya Uyguner, Ahmet Nayir, Sevgi Yavuz, Umut Altunoglu, Ozde Nisa Turkkan, Burcu Sevinc, Gulden Gokcay, Dilek Kurkcu Gunes, Aysel Kiyak, Alev Yilmaz. Primary coenzyme Q10 Deficiency-6 (COQ10D6): Two siblings with variable expressivity of the renal phenotype. European journal of medical genetics. 2020 Jan; 63(1):103621. doi: 10.1016/j.ejmg.2019.01.011. [PMID: 30682496]
  • Keita Nakanishi, Takayuki Okamoto, Kandai Nozu, Shigeo Hara, Yasuyuki Sato, Asako Hayashi, Toshiyuki Takahashi, China Nagano, Nana Sakakibara, Tomoko Horinouchi, Junya Fujimura, Shogo Minamikawa, Tomohiko Yamamura, Rini Rossanti, Hiroaki Nagase, Hiroshi Kaito, Tadashi Ariga, Kazumoto Iijima. Pair analysis and custom array CGH can detect a small copy number variation in COQ6 gene. Clinical and experimental nephrology. 2019 May; 23(5):669-675. doi: 10.1007/s10157-018-1682-z. [PMID: 30584653]
  • Manuel J Acosta Lopez, Eva Trevisson, Marcella Canton, Luis Vazquez-Fonseca, Valeria Morbidoni, Elisa Baschiera, Chiara Frasson, Ludovic Pelosi, Bérengère Rascalou, Maria Andrea Desbats, María Alcázar-Fabra, José Julián Ríos, Alicia Sánchez-García, Giuseppe Basso, Placido Navas, Fabien Pierrel, Gloria Brea-Calvo, Leonardo Salviati. Vanillic Acid Restores Coenzyme Q Biosynthesis and ATP Production in Human Cells Lacking COQ6. Oxidative medicine and cellular longevity. 2019; 2019(?):3904905. doi: 10.1155/2019/3904905. [PMID: 31379988]
  • Małgorzata Stańczyk, Irena Bałasz-Chmielewska, Beata Lipska-Ziętkiewicz, Marcin Tkaczyk. CoQ10-related sustained remission of proteinuria in a child with COQ6 glomerulopathy-a case report. Pediatric nephrology (Berlin, Germany). 2018 Dec; 33(12):2383-2387. doi: 10.1007/s00467-018-4083-3. [PMID: 30232548]
  • Cheng-Cheng Song, Quan Hong, Xiao-Dong Geng, Xu Wang, Shu-Qiang Wang, Shao-Yuan Cui, Man-Di Guo, Ou Li, Guang-Yan Cai, Xiang-Mei Chen, Di Wu. New Mutation of Coenzyme Q10 Monooxygenase 6 Causing Podocyte Injury in a Focal Segmental Glomerulosclerosis Patient. Chinese medical journal. 2018 Nov; 131(22):2666-2675. doi: 10.4103/0366-6999.245158. [PMID: 30425193]
  • Agape M Awad, Srivats Venkataramanan, Anish Nag, Anoop Raj Galivanche, Michelle C Bradley, Lauren T Neves, Stephen Douglass, Catherine F Clarke, Tracy L Johnson. Chromatin-remodeling SWI/SNF complex regulates coenzyme Q6 synthesis and a metabolic shift to respiration in yeast. The Journal of biological chemistry. 2017 09; 292(36):14851-14866. doi: 10.1074/jbc.m117.798397. [PMID: 28739803]
  • Jun-Yi Zhu, Yulong Fu, Adam Richman, Zhanzheng Zhao, Patricio E Ray, Zhe Han. A Personalized Model of COQ2 Nephropathy Rescued by the Wild-Type COQ2 Allele or Dietary Coenzyme Q10 Supplementation. Journal of the American Society of Nephrology : JASN. 2017 Sep; 28(9):2607-2617. doi: 10.1681/asn.2016060626. [PMID: 28428331]
  • Eujin Park, Yo Han Ahn, Hee Gyung Kang, Kee Hwan Yoo, Nam Hee Won, Kyoung Bun Lee, Kyung Chul Moon, Moon-Woo Seong, Tae Rin Gwon, Sung Sup Park, Hae Il Cheong. COQ6 Mutations in Children With Steroid-Resistant Focal Segmental Glomerulosclerosis and Sensorineural Hearing Loss. American journal of kidney diseases : the official journal of the National Kidney Foundation. 2017 Jul; 70(1):139-144. doi: 10.1053/j.ajkd.2016.10.040. [PMID: 28117207]
  • Q Cao, G M Li, H Xu, Q Shen, L Sun, X Y Fang, H M Liu, W Guo, Y H Zhai, B B Wu. [Coenzyme Q(10) treatment for one child with COQ6 gene mutation induced nephrotic syndrome and literature review]. Zhonghua er ke za zhi = Chinese journal of pediatrics. 2017 Feb; 55(2):135-138. doi: 10.3760/cma.j.issn.0578-1310.2017.02.016. [PMID: 28173653]
  • Alexandre Ismail, Vincent Leroux, Myriam Smadja, Lucie Gonzalez, Murielle Lombard, Fabien Pierrel, Caroline Mellot-Draznieks, Marc Fontecave. Coenzyme Q Biosynthesis: Evidence for a Substrate Access Channel in the FAD-Dependent Monooxygenase Coq6. PLoS computational biology. 2016 Jan; 12(1):e1004690. doi: 10.1371/journal.pcbi.1004690. [PMID: 26808124]
  • Mohammad Ozeir, Ludovic Pelosi, Alexandre Ismail, Caroline Mellot-Draznieks, Marc Fontecave, Fabien Pierrel. Coq6 is responsible for the C4-deamination reaction in coenzyme Q biosynthesis in Saccharomyces cerevisiae. The Journal of biological chemistry. 2015 Oct; 290(40):24140-51. doi: 10.1074/jbc.m115.675744. [PMID: 26260787]
  • Isabel González-Mariscal, Elena García-Testón, Sergio Padilla, Alejandro Martín-Montalvo, Teresa Pomares-Viciana, Luis Vazquez-Fonseca, Pablo Gandolfo-Domínguez, Carlos Santos-Ocaña. Regulation of coenzyme Q biosynthesis in yeast: a new complex in the block. IUBMB life. 2014 Feb; 66(2):63-70. doi: 10.1002/iub.1243. [PMID: 24470391]
  • Saskia F Heeringa, Gil Chernin, Moumita Chaki, Weibin Zhou, Alexis J Sloan, Ziming Ji, Letian X Xie, Leonardo Salviati, Toby W Hurd, Virginia Vega-Warner, Paul D Killen, Yehoash Raphael, Shazia Ashraf, Bugsu Ovunc, Dominik S Schoeb, Heather M McLaughlin, Rannar Airik, Christopher N Vlangos, Rasheed Gbadegesin, Bernward Hinkes, Pawaree Saisawat, Eva Trevisson, Mara Doimo, Alberto Casarin, Vanessa Pertegato, Gianpietro Giorgi, Holger Prokisch, Agnès Rötig, Gudrun Nürnberg, Christian Becker, Su Wang, Fatih Ozaltin, Rezan Topaloglu, Aysin Bakkaloglu, Sevcan A Bakkaloglu, Dominik Müller, Antje Beissert, Sevgi Mir, Afig Berdeli, Seza Varpizen, Martin Zenker, Verena Matejas, Carlos Santos-Ocaña, Placido Navas, Takehiro Kusakabe, Andreas Kispert, Sema Akman, Neveen A Soliman, Stefanie Krick, Peter Mundel, Jochen Reiser, Peter Nürnberg, Catherine F Clarke, Roger C Wiggins, Christian Faul, Friedhelm Hildebrandt. COQ6 mutations in human patients produce nephrotic syndrome with sensorineural deafness. The Journal of clinical investigation. 2011 May; 121(5):2013-24. doi: 10.1172/jci45693. [PMID: 21540551]
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  • Sergio Padilla-López, María Jiménez-Hidalgo, Alejandro Martín-Montalvo, Catherine F Clarke, Plácido Navas, Carlos Santos-Ocaña. Genetic evidence for the requirement of the endocytic pathway in the uptake of coenzyme Q6 in Saccharomyces cerevisiae. Biochimica et biophysica acta. 2009 Jun; 1788(6):1238-48. doi: 10.1016/j.bbamem.2009.03.018. [PMID: 19345667]
  • S Padilla, U C Tran, M Jiménez-Hidalgo, J M López-Martín, A Martín-Montalvo, C F Clarke, P Navas, C Santos-Ocaña. Hydroxylation of demethoxy-Q6 constitutes a control point in yeast coenzyme Q6 biosynthesis. Cellular and molecular life sciences : CMLS. 2009 Jan; 66(1):173-86. doi: 10.1007/s00018-008-8547-7. [PMID: 19002377]
  • Mei Zhang, Jun Luo, Yuki Ogiyama, Ryoichi Saiki, Makoto Kawamukai. Heteromer formation of a long-chain prenyl diphosphate synthase from fission yeast Dps1 and budding yeast Coq1. The FEBS journal. 2008 Jul; 275(14):3653-68. doi: 10.1111/j.1742-4658.2008.06510.x. [PMID: 18540885]
  • Beth Marbois, Peter Gin, Kym F Faull, Wayne W Poon, Peter T Lee, Jeff Strahan, Jennifer N Shepherd, Catherine F Clarke. Coq3 and Coq4 define a polypeptide complex in yeast mitochondria for the biosynthesis of coenzyme Q. The Journal of biological chemistry. 2005 May; 280(21):20231-8. doi: 10.1074/jbc.m501315200. [PMID: 15792955]
  • Peter Gin, Adam Y Hsu, Steven C Rothman, Tanya Jonassen, Peter T Lee, Alexander Tzagoloff, Catherine F Clarke. The Saccharomyces cerevisiae COQ6 gene encodes a mitochondrial flavin-dependent monooxygenase required for coenzyme Q biosynthesis. The Journal of biological chemistry. 2003 Jul; 278(28):25308-16. doi: 10.1074/jbc.m303234200. [PMID: 12721307]
  • S Mohan, C G Pradeep, R Abhilash Kumar, K Jayakumar, R Kaleysa Raj. The adult-specific ubiquinone Q(8) functions as an antioxidant in the filarial parasite, Setaria digitata. Biochemical and biophysical research communications. 2001 Nov; 288(4):949-53. doi: 10.1006/bbrc.2001.5882. [PMID: 11689001]
  • C Santos-Ocaña, F Córdoba, F L Crane, C F Clarke, P Navas. Coenzyme Q6 and iron reduction are responsible for the extracellular ascorbate stabilization at the plasma membrane of Saccharomyces cerevisiae. The Journal of biological chemistry. 1998 Apr; 273(14):8099-105. doi: 10.1074/jbc.273.14.8099. [PMID: 9525912]