Glycerol phenylbutyrate (BioDeep_00000179522)

   

human metabolite blood metabolite


代谢物信息卡片


1,3-Bis[(4-phenylbutanoyl)oxy]propan-2-yl 4-phenylbutanoic acid

化学式: C33H38O6 (530.2668248)
中文名称: 苯丁酸甘油酯
谱图信息: 最多检出来源 Homo sapiens(blood) 17.65%

分子结构信息

SMILES: C1=CC=C(C=C1)CCCC(=O)OCC(COC(=O)CCCC2=CC=CC=C2)OC(=O)CCCC3=CC=CC=C3
InChI: InChI=1S/C33H38O6/c34-31(22-10-19-27-13-4-1-5-14-27)37-25-30(39-33(36)24-12-21-29-17-8-3-9-18-29)26-38-32(35)23-11-20-28-15-6-2-7-16-28/h1-9,13-18,30H,10-12,19-26H2

描述信息

A - Alimentary tract and metabolism > A16 - Other alimentary tract and metabolism products > A16A - Other alimentary tract and metabolism products > A16AX - Various alimentary tract and metabolism products
C26170 - Protective Agent

同义名列表

7 个代谢物同义名

1,3-Bis[(4-phenylbutanoyl)oxy]propan-2-yl 4-phenylbutanoic acid; 1,3-bis[(4-phenylbutanoyl)oxy]propan-2-yl 4-phenylbutanoate; Glycerol phenylbutyric acid; Glycerol phenylbutyrate; HPN-100; HPN 100; Ravicti



数据库引用编号

7 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)

1 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

  • Nicola Longo, George A Diaz, Uta Lichter-Konecki, Andreas Schulze, Michal Inbar-Feigenberg, Robert L Conway, Allison A Bannick, Shawn E McCandless, Roberto Zori, Bryan Hainline, Nicholas Ah Mew, Colleen Canavan, Thomas Vescio, Teresa Kok, Marty H Porter, Susan A Berry. Glycerol phenylbutyrate efficacy and safety from an open label study in pediatric patients under 2 months of age with urea cycle disorders. Molecular genetics and metabolism. 2021 01; 132(1):19-26. doi: 10.1016/j.ymgme.2020.12.002. [PMID: 33388234]
  • Roland Posset, Andrea L Gropman, Sandesh C S Nagamani, Lindsay C Burrage, Jirair K Bedoyan, Derek Wong, Gerard T Berry, Matthias R Baumgartner, Marc Yudkoff, Matthias Zielonka, Georg F Hoffmann, Peter Burgard, Andreas Schulze, Shawn E McCandless, Angeles Garcia-Cazorla, Jennifer Seminara, Sven F Garbade, Stefan Kölker. Impact of Diagnosis and Therapy on Cognitive Function in Urea Cycle Disorders. Annals of neurology. 2019 07; 86(1):116-128. doi: 10.1002/ana.25492. [PMID: 31018246]
  • Susan A Berry, Jerry Vockley, Alexander A Vinks, Min Dong, George A Diaz, Shawn E McCandless, Wendy E Smith, Cary O Harding, Roberto Zori, Can Ficicioglu, Uta Lichter-Konecki, Renee Perdok, Beth Robinson, Robert J Holt, Nicola Longo. Pharmacokinetics of glycerol phenylbutyrate in pediatric patients 2 months to 2 years of age with urea cycle disorders. Molecular genetics and metabolism. 2018 11; 125(3):251-257. doi: 10.1016/j.ymgme.2018.09.001. [PMID: 30217721]
  • Yi Jiang, Mohammed Almannai, V Reid Sutton, Qin Sun, Sarah H Elsea. Quantitation of phenylbutyrate metabolites by UPLC-MS/MS demonstrates inverse correlation of phenylacetate:phenylacetylglutamine ratio with plasma glutamine levels. Molecular genetics and metabolism. 2017 11; 122(3):39-45. doi: 10.1016/j.ymgme.2017.08.011. [PMID: 28888854]
  • Sandesh C S Nagamani, George A Diaz, William Rhead, Susan A Berry, Cynthia Le Mons, Uta Lichter-Konecki, James Bartley, Annette Feigenbaum, Andreas Schulze, Nicola Longo, William Berquist, Renata Gallagher, Dennis Bartholomew, Cary O Harding, Mark S Korson, Shawn E McCandless, Wendy Smith, Jerry Vockley, David Kronn, Robert Zori, Stephen Cederbaum, J Lawrence Merritt, Derek Wong, Dion F Coakley, Bruce F Scharschmidt, Klara Dickinson, Miguel Marino, Brendan H Lee, Masoud Mokhtarani. Self-reported treatment-associated symptoms among patients with urea cycle disorders participating in glycerol phenylbutyrate clinical trials. Molecular genetics and metabolism. 2015 Sep; 116(1-2):29-34. doi: 10.1016/j.ymgme.2015.08.002. [PMID: 26296711]
  • Don C Rockey, John M Vierling, Parvez Mantry, Marwan Ghabril, Robert S Brown, Olga Alexeeva, Igor A Zupanets, Vladimir Grinevich, Andrey Baranovsky, Larysa Dudar, Galyna Fadieienko, Nataliya Kharchenko, Iryna Klaryts'ka, Vyacheslav Morozov, Priya Grewal, Timothy McCashland, K Gautham Reddy, K Rajender Reddy, Vasyl Syplyviy, Nathan M Bass, Klara Dickinson, Catherine Norris, Dion Coakley, Masoud Mokhtarani, Bruce F Scharschmidt. Randomized, double-blind, controlled study of glycerol phenylbutyrate in hepatic encephalopathy. Hepatology (Baltimore, Md.). 2014 Mar; 59(3):1073-83. doi: 10.1002/hep.26611. [PMID: 23847109]
  • Juan Córdoba, Meritxell Ventura-Cots. Drug-induced removal of nitrogen derivatives in urine: a new concept whose time has come. Hepatology (Baltimore, Md.). 2014 Mar; 59(3):764-6. doi: 10.1002/hep.26789. [PMID: 24123173]
  • M Mokhtarani, G A Diaz, W Rhead, S A Berry, U Lichter-Konecki, A Feigenbaum, A Schulze, N Longo, J Bartley, W Berquist, R Gallagher, W Smith, S E McCandless, C Harding, D C Rockey, J M Vierling, P Mantry, M Ghabril, R S Brown, K Dickinson, T Moors, C Norris, D Coakley, D A Milikien, S C Nagamani, C Lemons, B Lee, B F Scharschmidt. Elevated phenylacetic acid levels do not correlate with adverse events in patients with urea cycle disorders or hepatic encephalopathy and can be predicted based on the plasma PAA to PAGN ratio. Molecular genetics and metabolism. 2013 Dec; 110(4):446-53. doi: 10.1016/j.ymgme.2013.09.017. [PMID: 24144944]
  • Jon P R Monteleone, M Mokhtarani, G A Diaz, W Rhead, U Lichter-Konecki, S A Berry, C Lemons, K Dickinson, D Coakley, B Lee, B F Scharschmidt. Population pharmacokinetic modeling and dosing simulations of nitrogen-scavenging compounds: disposition of glycerol phenylbutyrate and sodium phenylbutyrate in adult and pediatric patients with urea cycle disorders. Journal of clinical pharmacology. 2013 Jul; 53(7):699-710. doi: 10.1002/jcph.92. [PMID: 23775211]
  • Wendy Smith, George A Diaz, Uta Lichter-Konecki, Susan A Berry, Cary O Harding, Shawn E McCandless, Cindy LeMons, Joe Mauney, Klara Dickinson, Dion F Coakley, Tristen Moors, Masoud Mokhtarani, Bruce F Scharschmidt, Brendan Lee. Ammonia control in children ages 2 months through 5 years with urea cycle disorders: comparison of sodium phenylbutyrate and glycerol phenylbutyrate. The Journal of pediatrics. 2013 Jun; 162(6):1228-34, 1234.e1. doi: 10.1016/j.jpeds.2012.11.084. [PMID: 23324524]
  • George A Diaz, Lauren S Krivitzky, Masoud Mokhtarani, William Rhead, James Bartley, Annette Feigenbaum, Nicola Longo, William Berquist, Susan A Berry, Renata Gallagher, Uta Lichter-Konecki, Dennis Bartholomew, Cary O Harding, Stephen Cederbaum, Shawn E McCandless, Wendy Smith, Gerald Vockley, Stephen A Bart, Mark S Korson, David Kronn, Roberto Zori, J Lawrence Merritt, Sandesh C S Nagamani, Joseph Mauney, Cynthia Lemons, Klara Dickinson, Tristen L Moors, Dion F Coakley, Bruce F Scharschmidt, Brendan Lee. Ammonia control and neurocognitive outcome among urea cycle disorder patients treated with glycerol phenylbutyrate. Hepatology (Baltimore, Md.). 2013 Jun; 57(6):2171-9. doi: 10.1002/hep.26058. [PMID: 22961727]
  • M Mokhtarani, G A Diaz, W Rhead, U Lichter-Konecki, J Bartley, A Feigenbaum, N Longo, W Berquist, S A Berry, R Gallagher, D Bartholomew, C O Harding, M S Korson, S E McCandless, W Smith, J Vockley, S Bart, D Kronn, R Zori, S Cederbaum, N Dorrani, J L Merritt, Sandesh Sreenath-Nagamani, M Summar, C Lemons, K Dickinson, D F Coakley, T L Moors, B Lee, B F Scharschmidt. Urinary phenylacetylglutamine as dosing biomarker for patients with urea cycle disorders. Molecular genetics and metabolism. 2012 Nov; 107(3):308-14. doi: 10.1016/j.ymgme.2012.08.006. [PMID: 22958974]
  • Uta Lichter-Konecki, G A Diaz, J L Merritt, A Feigenbaum, C Jomphe, J F Marier, M Beliveau, J Mauney, K Dickinson, A Martinez, M Mokhtarani, B Scharschmidt, W Rhead. Ammonia control in children with urea cycle disorders (UCDs); phase 2 comparison of sodium phenylbutyrate and glycerol phenylbutyrate. Molecular genetics and metabolism. 2011 Aug; 103(4):323-9. doi: 10.1016/j.ymgme.2011.04.013. [PMID: 21612962]