Pristanate (BioDeep_00000023963)

   

human metabolite Endogenous blood metabolite


代谢物信息卡片


2,6,10,14-tetramethylpentadecanoic acid

化学式: C19H38O2 (298.2871648)
中文名称: 降植烷酸
谱图信息: 最多检出来源 Homo sapiens(blood) 2.29%

Reviewed

Last reviewed on 2024-09-14.

Cite this Page

Pristanate. BioDeep Database v3. PANOMIX ltd, a top metabolomics service provider from China. https://query.biodeep.cn/s/pristanate (retrieved 2024-11-10) (BioDeep RN: BioDeep_00000023963). Licensed under the Attribution-Noncommercial 4.0 International License (CC BY-NC 4.0).

分子结构信息

SMILES: CC(C)CCCC(C)CCCC(C)CCCC(C)C(=O)O
InChI: InChI=1S/C19H38O2/c1-15(2)9-6-10-16(3)11-7-12-17(4)13-8-14-18(5)19(20)21/h15-18H,6-14H2,1-5H3,(H,20,21)

描述信息

Pristanic acid (2,6,10,14-tetramethylpentadecanoic acid) is a terpenoid acid present at micromolar concentrations in the plasma of healthy individuals. It is also found in the lipids from many sources such as freshwater sponges, krill, earthworms, whales, human milk fat, bovine depot fat, butterfat or Californian petroleum. It is usually present in combination with phytanic acid. In humans, pristanic acid is obtained from two sources: either directly from the diet or as the alpha oxidation product of phytanic acid. At physiological concentrations pristanic acid is a natural ligand for PPARalpha. In liver, pristanic acid is degraded by peroxisomal beta oxidation to propionyl-CoA. Together with phytanic acid, pristanic acid accumulates in several inherited disorders such as Zellweger syndrome. Pristanic acid is a branched chain fatty acid that arises from the breakdown of phytanic acid. It is present at micromolar concentrations in the plasma of healthy individuals. Pristanic acid is normally degraded by peroxisomal beta-oxidation. In patients affected with generalized peroxisomal disorders, degradation of both phytanic acid and pristanic acid is impaired owing to absence of functional peroxisomes. Pristanic acid has been found to activate the peroxisome proliferator-activated receptor {alpha} (PPAR{alpha}) in a concentration dependent manner.
Component of butterfat and sheep perinephric fat

同义名列表

15 个代谢物同义名

(2S,6R,10R)-2,6,10,14-Tetramethylpentadecanoic acid; (2S,6R,10R)-2,6,10,14-Tetramethylpentadecanoate; 2,6,10,14-Tetramethylpentadecanoic acid; 2,6,10,14-Tetramethylpentadecylic acid; 2,6,10,14-Tetramethyl-pentadecansaeure; 2,6,10,14-Tetramethylpentadecanoate; 2,6,10,14-Tetramethylpentadecylate; Pristanate:(2S,6R,10R)-pristanate; (2S,6R,10R)-Pristanic acid; (2S)-Pristanic acid; Acide pristanique; Acido pristanico; Pristaninsaeure; pristanic acid; Pristanate



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(0)

BioCyc(0)

PlantCyc(0)

代谢反应

15 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(15)

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

  • Maaike Blankestijn, Vincent W Bloks, Dicky Struik, Nicolette Huijkman, Niels Kloosterhuis, Justina C Wolters, Ronald J A Wanders, Frédéric M Vaz, Markus Islinger, Folkert Kuipers, Bart van de Sluis, Albert K Groen, Henkjan J Verkade, Johan W Jonker. Mice with a deficiency in Peroxisomal Membrane Protein 4 (PXMP4) display mild changes in hepatic lipid metabolism. Scientific reports. 2022 02; 12(1):2512. doi: 10.1038/s41598-022-06479-y. [PMID: 35169201]
  • Tomonori Nakanishi, Kazuhiro Kagamizono, Sayaka Yokoyama, Ryoji Suzuki, Hiroyuki Sakakibara, Laurie Erickson, Satoshi Kawahara. Effects of dietary phytol on tissue accumulation of phytanic acid and pristanic acid and on the tissue lipid profiles in mice. Animal science journal = Nihon chikusan Gakkaiho. 2020 Jan; 91(1):e13424. doi: 10.1111/asj.13424. [PMID: 32618084]
  • Serena Mezzar, Evelyn De Schryver, Stanny Asselberghs, Els Meyhi, Petruta L Morvay, Myriam Baes, Paul P Van Veldhoven. Phytol-induced pathology in 2-hydroxyacyl-CoA lyase (HACL1) deficient mice. Evidence for a second non-HACL1-related lyase. Biochimica et biophysica acta. Molecular and cell biology of lipids. 2017 Sep; 1862(9):972-990. doi: 10.1016/j.bbalip.2017.06.004. [PMID: 28629946]
  • Katharina Herzog, Henk van Lenthe, Ronald J A Wanders, Frédéric M Vaz, Hans R Waterham, Sacha Ferdinandusse. Identification and diagnostic value of phytanoyl- and pristanoyl-carnitine in plasma from patients with peroxisomal disorders. Molecular genetics and metabolism. 2017 07; 121(3):279-282. doi: 10.1016/j.ymgme.2017.05.003. [PMID: 28566232]
  • Michela Semeraro, Cristiano Rizzo, Sara Boenzi, Marco Cappa, Enrico Bertini, Giacomo Antonetti, Carlo Dionisi-Vici. A new multiplex method for the diagnosis of peroxisomal disorders allowing simultaneous determination of plasma very-long-chain fatty acids, phytanic, pristanic, docosahexaenoic and bile acids by high-performance liquid chromatography-atmospheric pressure chemical ionization-tandem mass spectrometry. Clinica chimica acta; international journal of clinical chemistry. 2016 Jul; 458(?):159-64. doi: 10.1016/j.cca.2016.05.009. [PMID: 27189059]
  • Arthur Sorlin, Gilbert Briand, David Cheillan, Arnaud Wiedemann, Bettina Montaut-Verient, Emmanuelle Schmitt, François Feillet. Effect of l-Arginine in One Patient with Peroxisome Biogenesis Disorder due to PEX12 Deficiency. Neuropediatrics. 2016 Jun; 47(3):179-81. doi: 10.1055/s-0036-1578798. [PMID: 26947510]
  • Sacha Ferdinandusse, Gerardo Jimenez-Sanchez, Janet Koster, Simone Denis, Carlo W Van Roermund, Irma Silva-Zolezzi, Ann B Moser, Wouter F Visser, Mine Gulluoglu, Ozlem Durmaz, Mubeccel Demirkol, Hans R Waterham, Gülden Gökcay, Ronald J A Wanders, David Valle. A novel bile acid biosynthesis defect due to a deficiency of peroxisomal ABCD3. Human molecular genetics. 2015 Jan; 24(2):361-70. doi: 10.1093/hmg/ddu448. [PMID: 25168382]
  • Estela Natacha Brandt Busanello, Vannessa Gonçalves Araujo Lobato, Ângela Zanatta, Clarissa Günther Borges, Anelise Miotti Tonin, Carolina Maso Viegas, Vanusa Manfredini, César Augusto João Ribeiro, Carmen Regla Vargas, Diogo Onofre Gomes de Souza, Moacir Wajner. Pristanic acid provokes lipid, protein, and DNA oxidative damage and reduces the antioxidant defenses in cerebellum of young rats. Cerebellum (London, England). 2014 Dec; 13(6):751-9. doi: 10.1007/s12311-014-0593-0. [PMID: 25172216]
  • Margaret E Wright, Demetrius Albanes, Ann B Moser, Stephanie J Weinstein, Kirk Snyder, Satu Männistö, Peter H Gann. Serum phytanic and pristanic acid levels and prostate cancer risk in Finnish smokers. Cancer medicine. 2014 Dec; 3(6):1562-9. doi: 10.1002/cam4.319. [PMID: 25132681]
  • Catherine J Klein, Thomas G Havranek, Mary E Revenis, Zahra Hassanali, Louis M Scavo. Plasma fatty acids in premature infants with hyperbilirubinemia: before-and-after nutrition support with fish oil emulsion. Nutrition in clinical practice : official publication of the American Society for Parenteral and Enteral Nutrition. 2013 Feb; 28(1):87-94. doi: 10.1177/0884533612469989. [PMID: 23319354]
  • Hugh J McMillan, Thea Worthylake, Jeremy Schwartzentruber, Chloe C Gottlieb, Sarah E Lawrence, Alex Mackenzie, Chandree L Beaulieu, Petra A W Mooyer, Ronald J A Wanders, Jacek Majewski, Dennis E Bulman, Michael T Geraghty, Sacha Ferdinandusse, Kym M Boycott. Specific combination of compound heterozygous mutations in 17β-hydroxysteroid dehydrogenase type 4 (HSD17B4) defines a new subtype of D-bifunctional protein deficiency. Orphanet journal of rare diseases. 2012 Nov; 7(?):90. doi: 10.1186/1750-1172-7-90. [PMID: 23181892]
  • I S Mamedov, Iu A Smolina, V S Sukhorukov, P V Novikov. [The diagnostic of peroxisomic diseases in children]. Klinicheskaia laboratornaia diagnostika. 2012 Mar; ?(3):16-8. doi: NULL. [PMID: 22712285]
  • Estela Natacha Brandt Busanello, Alexandre Umpierrez Amaral, Anelise Miotti Tonin, Mateus Grings, Alana Pimentel Moura, Paula Eichler, Carmen Regla Vargas, Moacir Wajner. Experimental evidence that pristanic acid disrupts mitochondrial homeostasis in brain of young rats. Journal of neuroscience research. 2012 Mar; 90(3):597-605. doi: 10.1002/jnr.22802. [PMID: 22183871]
  • Jianqiu Kou, Gabor G Kovacs, Romana Höftberger, Willem Kulik, Alexander Brodde, Sonja Forss-Petter, Selma Hönigschnabl, Andreas Gleiss, Britta Brügger, Ronald Wanders, Wilhelm Just, Herbert Budka, Susanne Jungwirth, Peter Fischer, Johannes Berger. Peroxisomal alterations in Alzheimer's disease. Acta neuropathologica. 2011 Sep; 122(3):271-83. doi: 10.1007/s00401-011-0836-9. [PMID: 21594711]
  • Gisella Terre'Blanche, Mietha M van der Walt, Jacobus J Bergh, Lodewyk J Mienie. Treatment of an adrenomyeloneuropathy patient with Lorenzo's oil and supplementation with docosahexaenoic acid--a case report. Lipids in health and disease. 2011 Aug; 10(?):152. doi: 10.1186/1476-511x-10-152. [PMID: 21871076]
  • Nicol Kruska, Georg Reiser. Phytanic acid and pristanic acid, branched-chain fatty acids associated with Refsum disease and other inherited peroxisomal disorders, mediate intracellular Ca2+ signaling through activation of free fatty acid receptor GPR40. Neurobiology of disease. 2011 Aug; 43(2):465-72. doi: 10.1016/j.nbd.2011.04.020. [PMID: 21570468]
  • Louise B Werner, Lars I Hellgren, Marianne Raff, Søren K Jensen, Rikke A Petersen, Tue Drachmann, Tine Tholstrup. Effect of dairy fat on plasma phytanic acid in healthy volunteers - a randomized controlled study. Lipids in health and disease. 2011 Jun; 10(?):95. doi: 10.1186/1476-511x-10-95. [PMID: 21663648]
  • William B Rizzo. The role of fatty aldehyde dehydrogenase in epidermal structure and function. Dermato-endocrinology. 2011 Apr; 3(2):91-9. doi: 10.4161/derm.3.2.14619. [PMID: 21695018]
  • Guilhian Leipnitz, Alexandre Umpierrez Amaral, Carolina Gonçalves Fernandes, Bianca Seminotti, Angela Zanatta, Lisiane Aurélio Knebel, Carmen Regla Vargas, Moacir Wajner. Pristanic acid promotes oxidative stress in brain cortex of young rats: a possible pathophysiological mechanism for brain damage in peroxisomal disorders. Brain research. 2011 Mar; 1382(?):259-65. doi: 10.1016/j.brainres.2011.01.014. [PMID: 21241675]
  • Emily Helen Smith, Dimitar K Gavrilov, Devin Oglesbee, William D Freeman, Michael W Vavra, Dietrich Matern, Silvia Tortorelli. An adult onset case of alpha-methyl-acyl-CoA racemase deficiency. Journal of inherited metabolic disease. 2010 Dec; 33 Suppl 3(?):S349-53. doi: 10.1007/s10545-010-9183-6. [PMID: 20821052]
  • Paul A Watkins, Ann B Moser, Cicely B Toomer, Steven J Steinberg, Hugo W Moser, Mazen W Karaman, Krishna Ramaswamy, Kimberly D Siegmund, D Rick Lee, John J Ely, Oliver A Ryder, Joseph G Hacia. Identification of differences in human and great ape phytanic acid metabolism that could influence gene expression profiles and physiological functions. BMC physiology. 2010 Oct; 10(?):19. doi: 10.1186/1472-6793-10-19. [PMID: 20932325]
  • Ronald J A Wanders, Jos P N Ruiter, Lodewijk IJLst, Hans R Waterham, Sander M Houten. The enzymology of mitochondrial fatty acid beta-oxidation and its application to follow-up analysis of positive neonatal screening results. Journal of inherited metabolic disease. 2010 Oct; 33(5):479-94. doi: 10.1007/s10545-010-9104-8. [PMID: 20490924]
  • Sean R Pyper, Navin Viswakarma, Songtao Yu, Janardan K Reddy. PPARalpha: energy combustion, hypolipidemia, inflammation and cancer. Nuclear receptor signaling. 2010 Apr; 8(?):e002. doi: 10.1621/nrs.08002. [PMID: 20414453]
  • Lars I Hellgren. Phytanic acid--an overlooked bioactive fatty acid in dairy fat?. Annals of the New York Academy of Sciences. 2010 Mar; 1190(?):42-9. doi: 10.1111/j.1749-6632.2009.05254.x. [PMID: 20388135]
  • Cindy Krause, Hendrik Rosewich, Jutta Gärtner. Rational diagnostic strategy for Zellweger syndrome spectrum patients. European journal of human genetics : EJHG. 2009 Jun; 17(6):741-8. doi: 10.1038/ejhg.2008.252. [PMID: 19142205]
  • John T Mackie, Barbara P Atshaves, H Ross Payne, Avery L McIntosh, Friedhelm Schroeder, Ann B Kier. Phytol-induced hepatotoxicity in mice. Toxicologic pathology. 2009 Feb; 37(2):201-8. doi: 10.1177/0192623308330789. [PMID: 19188468]
  • T Fiskerstrand, P Knappskog, J Majewski, R J Wanders, H Boman, L A Bindoff. A novel Refsum-like disorder that maps to chromosome 20. Neurology. 2009 Jan; 72(1):20-7. doi: 10.1212/01.wnl.0000333664.90605.23. [PMID: 19005174]
  • Sian A Thompson, Jacqui Calvin, Sarah Hogg, Sacha Ferdinandusse, Ronald J A Wanders, Roger A Barker. Relapsing encephalopathy in a patient with α-methylacyl-CoA racemase deficiency. BMJ case reports. 2009; 2009(?):. doi: 10.1136/bcr.08.2008.0814. [PMID: 21686617]
  • Osama Y Al-Dirbashi, Tomofumi Santa, Mohamed S Rashed, Zuhair Al-Hassnan, Nobuyuki Shimozawa, Aziza Chedrawi, Minnie Jacob, Manhal Al-Mokhadab. Rapid UPLC-MS/MS method for routine analysis of plasma pristanic, phytanic, and very long chain fatty acid markers of peroxisomal disorders. Journal of lipid research. 2008 Aug; 49(8):1855-62. doi: 10.1194/jlr.d800019-jlr200. [PMID: 18441019]
  • Rosalba Carrozzo, Carlo Bellini, Simona Lucioli, Federica Deodato, Denise Cassandrini, Michela Cassanello, Ubaldo Caruso, Cristiano Rizzo, Teresa Rizza, Matteo L Napolitano, Ronald J A Wanders, Cornelis Jakobs, Claudio Bruno, Filippo M Santorelli, Carlo Dionisi-Vici, Eugenio Bonioli. Peroxisomal acyl-CoA-oxidase deficiency: two new cases. American journal of medical genetics. Part A. 2008 Jul; 146A(13):1676-81. doi: 10.1002/ajmg.a.32298. [PMID: 18536048]
  • S A Thompson, J Calvin, S Hogg, S Ferdinandusse, R J A Wanders, R A Barker. Relapsing encephalopathy in a patient with alpha-methylacyl-CoA racemase deficiency. Journal of neurology, neurosurgery, and psychiatry. 2008 Apr; 79(4):448-50. doi: 10.1136/jnnp.2007.129478. [PMID: 18032455]
  • Bing Song Zheng, Elin Rönnberg, Lenita Viitanen, Tiina A Salminen, Krister Lundgren, Thomas Moritz, Johan Edqvist. Arabidopsis sterol carrier protein-2 is required for normal development of seeds and seedlings. Journal of experimental botany. 2008; 59(12):3485-99. doi: 10.1093/jxb/ern201. [PMID: 18687588]
  • Barbara P Atshaves, Avery L McIntosh, Danilo Landrock, H Ross Payne, John T Mackie, Nobuyo Maeda, Judith Ball, Friedhelm Schroeder, Ann B Kier. Effect of SCP-x gene ablation on branched-chain fatty acid metabolism. American journal of physiology. Gastrointestinal and liver physiology. 2007 Mar; 292(3):G939-51. doi: 10.1152/ajpgi.00308.2006. [PMID: 17068117]
  • J Gloerich, D M van den Brink, J P N Ruiter, N van Vlies, F M Vaz, R J A Wanders, S Ferdinandusse. Metabolism of phytol to phytanic acid in the mouse, and the role of PPARalpha in its regulation. Journal of lipid research. 2007 Jan; 48(1):77-85. doi: 10.1194/jlr.m600050-jlr200. [PMID: 17015885]
  • Francesc Villarroya, Roser Iglesias, Marta Giralt. PPARs in the Control of Uncoupling Proteins Gene Expression. PPAR research. 2007; 2007(?):74364. doi: 10.1155/2007/74364. [PMID: 17389766]
  • Steven Huyghe, Henning Schmalbruch, Karel De Gendt, Guido Verhoeven, Florian Guillou, Paul P Van Veldhoven, Myriam Baes. Peroxisomal multifunctional protein 2 is essential for lipid homeostasis in Sertoli cells and male fertility in mice. Endocrinology. 2006 May; 147(5):2228-36. doi: 10.1210/en.2005-1571. [PMID: 16484321]
  • H Rosewich, H R Waterham, R J A Wanders, S Ferdinandusse, M Henneke, D Hunneman, J Gärtner. Pitfall in metabolic screening in a patient with fatal peroxisomal beta-oxidation defect. Neuropediatrics. 2006 Apr; 37(2):95-8. doi: 10.1055/s-2006-923943. [PMID: 16773508]
  • D S Roe, B Z Yang, C Vianey-Saban, E Struys, L Sweetman, C R Roe. Differentiation of long-chain fatty acid oxidation disorders using alternative precursors and acylcarnitine profiling in fibroblasts. Molecular genetics and metabolism. 2006 Jan; 87(1):40-7. doi: 10.1016/j.ymgme.2005.09.018. [PMID: 16297647]
  • J Gloerich, N van Vlies, G A Jansen, S Denis, J P N Ruiter, M A van Werkhoven, M Duran, F M Vaz, R J A Wanders, S Ferdinandusse. A phytol-enriched diet induces changes in fatty acid metabolism in mice both via PPARalpha-dependent and -independent pathways. Journal of lipid research. 2005 Apr; 46(4):716-26. doi: 10.1194/jlr.m400337-jlr200. [PMID: 15654129]
  • Barbara P Atshaves, Avery L McIntosh, H Ross Payne, John Mackie, Ann B Kier, Friedhelm Schroeder. Effect of branched-chain fatty acid on lipid dynamics in mice lacking liver fatty acid binding protein gene. American journal of physiology. Cell physiology. 2005 Mar; 288(3):C543-58. doi: 10.1152/ajpcell.00359.2004. [PMID: 15692150]
  • Gerold Mönnig, Johannes Wiekowski, Paulus Kirchhof, Jörg Stypmann, Gabriele Plenz, Larissa Fabritz, Hans-Jürgen Bruns, Lars Eckardt, Gerd Assmann, Wilhelm Haverkamp, Günter Breithardt, Udo Seedorf. Phytanic acid accumulation is associated with conduction delay and sudden cardiac death in sterol carrier protein-2/sterol carrier protein-x deficient mice. Journal of cardiovascular electrophysiology. 2004 Nov; 15(11):1310-6. doi: 10.1046/j.1540-8167.2004.03679.x. [PMID: 15574183]
  • C E Clarke, S Alger, M A Preece, M A Burdon, S Chavda, S Denis, S Ferdinandusse, R J A Wanders. Tremor and deep white matter changes in alpha-methylacyl-CoA racemase deficiency. Neurology. 2004 Jul; 63(1):188-9. doi: 10.1212/01.wnl.0000132841.81250.b7. [PMID: 15249642]
  • Maria A K Westin, Stefan E H Alexson, Mary C Hunt. Molecular cloning and characterization of two mouse peroxisome proliferator-activated receptor alpha (PPARalpha)-regulated peroxisomal acyl-CoA thioesterases. The Journal of biological chemistry. 2004 May; 279(21):21841-8. doi: 10.1074/jbc.m313863200. [PMID: 15007068]
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  • Mridul Mukherji, Christopher J Schofield, Anthony S Wierzbicki, Gerbert A Jansen, Ronald J A Wanders, Matthew D Lloyd. The chemical biology of branched-chain lipid metabolism. Progress in lipid research. 2003 Sep; 42(5):359-76. doi: 10.1016/s0163-7827(03)00016-x. [PMID: 12814641]
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  • N M Verhoeven, D S Schor, E A Struys, E E Jansen, ten Brink HJ, R J Wanders, C Jakobs. Analysis of pristanic acid beta-oxidation intermediates in plasma from healthy controls and patients affected with peroxisomal disorders by stable isotope dilution gas chromatography mass spectrometry. Journal of lipid research. 1999 Feb; 40(2):260-6. doi: 10.1016/s0022-2275(20)33365-4. [PMID: 9925655]
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