Adenylsuccinic acid (BioDeep_00000003347)

 

Secondary id: BioDeep_00000175745

human metabolite Endogenous Volatile Flavor Compounds natural product


代谢物信息卡片


(2S)-2-({9-[(2R,3R,4S,5R)-3,4-dihydroxy-5-[(phosphonooxy)methyl]oxolan-2-yl]-9H-purin-6-yl}amino)butanedioic acid

化学式: C14H18N5O11P (463.0740408)
中文名称: 腺苷酸基琥珀酸
谱图信息: 最多检出来源 Homo sapiens(natural_products) 0.53%

分子结构信息

SMILES: C([C@@H](C(=O)O)Nc1c2c(ncn1)n(cn2)[C@H]1[C@@H]([C@@H]([C@@H](COP(=O)(O)O)O1)O)O)C(=O)O
InChI: InChI=1S/C14H18N5O11P/c20-7(21)1-5(14(24)25)18-11-8-12(16-3-15-11)19(4-17-8)13-10(23)9(22)6(30-13)2-29-31(26,27)28/h3-6,9-10,13,22-23H,1-2H2,(H,20,21)(H,24,25)(H,15,16,18)(H2,26,27,28)/t5-,6+,9+,10+,13+/m0/s1

描述信息

Adenylsuccinic acid, also known as adenylosuccinate, succinyladenosine or aspartyl adenylate, belongs to the class of organic compounds known as purine ribonucleoside monophosphates. These are nucleotides consisting of a purine base linked to a ribose to which one monophosphate group is attached. Adenylsuccinic acid is found in all living organisms, ranging from bacteria to plants to animals. Adenylsuccinic acid is an important intermediate in the de novo purine biosynthesis pathway. Specifically, adenylsuccinic acid is an intermediate in the interconversion of purine nucleotides inosine monophosphate (IMP) and adenosine monophosphate (AMP). The enzyme adenylosuccinate synthase carries out the reaction by the addition of aspartate to IMP. This reaction requires the input of energy from a phosphoanhydride bond in the form of guanosine triphosphate (GTP). Adenylsuccinic acid is a substrate least one other important metabolic reaction in purine biosynthesis. In particular, adenylsuccinic acid can be converted into fumaric acid through its interaction with the enzyme known as adenylosuccinate lyase (or adenylosuccinase). Adenylosuccinate lyase deficiency, is a rare autosomal recessive metabolic disorder characterized by the appearance of succinylaminoimidazolecarboxamide riboside (SAICA riboside) and adenylsuccinic acid in cerebrospinal fluid and urine (PMID: 8412002). Adenylosuccinate lyase deficiency presents with varying degrees of psychomotor retardation, autism, muscle wasting, and epilepsy. The exact cause of the symptoms is unknown, but possibilities include not enough purine nucleotide synthesis for cell replication, malfunctioning of the purine nucleotide cycle, and a buildup of substrates to toxic levels.
Adenylsuccinic acid is a substrate of the enzyme adenylosuccinase [EC 4.3.2.2] in purine metabolism pathway. The accumulation of adenylsuccinic acid in body fluids occurs due to a deficiency of adenylosuccinase. (KEGG; PMID 8412002) [HMDB]
D018377 - Neurotransmitter Agents > D018846 - Excitatory Amino Acids
KEIO_ID A037; [MS2] KO008839
KEIO_ID A037; [MS3] KO008840
KEIO_ID A037

同义名列表

17 个代谢物同义名

(2S)-2-({9-[(2R,3R,4S,5R)-3,4-dihydroxy-5-[(phosphonooxy)methyl]oxolan-2-yl]-9H-purin-6-yl}amino)butanedioic acid; N-[9-(5-O-Phosphono-beta-D-ribofuranosyl)-9H-purin-6-yl]-L-aspartic acid; N-[9-(5-O-Phosphono-β-D-ribofuranosyl)-9H-purin-6-yl]-L-aspartic acid; N-[9-(5-O-Phosphono-beta-D-ribofuranosyl)-9H-purin-6-yl]aspartic acid; N-[9-(5-O-Phosphono-β-D-ribofuranosyl)-9H-purin-6-yl]aspartic acid; Succinyladenosine monophosphoric acid; Succinyladenosine 5’-monophosphate; Succinyladenosine 5-monophosphate; 6-Succino-5’-adenylic acid; N6-(1,2-Dicarboxyethyl)AMP; 6-Succino-5-adenylic acid; Adenylosuccinic acid; Adenylsuccinic acid; Aspartyl adenylate; Adenylosuccinate; Succinyl AMP; N6-(1,2-Dicarboxyethyl)-AMP



数据库引用编号

43 个数据库交叉引用编号

分类词条

相关代谢途径

Reactome(0)

BioCyc(5)

PlantCyc(0)

代谢反应

14 个相关的代谢反应过程信息。

Reactome(0)

BioCyc(6)

WikiPathways(0)

Plant Reactome(0)

INOH(3)

PlantCyc(0)

COVID-19 Disease Map(0)

PathBank(5)

PharmGKB(0)

10 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

  • Cara A Timpani, Craig A Goodman, Christos G Stathis, Jason D White, Kamel Mamchaoui, Gillian Butler-Browne, Nuri Gueven, Alan Hayes, Emma Rybalka. Adenylosuccinic acid therapy ameliorates murine Duchenne Muscular Dystrophy. Scientific reports. 2020 01; 10(1):1125. doi: 10.1038/s41598-020-57610-w. [PMID: 31980663]
  • Belén Pérez-Dueñas, Angela Sempere, Jaume Campistol, Itziar Alonso-Colmenero, María Díez, Verónica González, Begoña Merinero, Lourdes R Desviat, Rafael Artuch. Novel features in the evolution of adenylosuccinate lyase deficiency. European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology Society. 2012 Jul; 16(4):343-8. doi: 10.1016/j.ejpn.2011.08.008. [PMID: 21903433]
  • Fahimeh Alipour Khesht, Ahmad Hassanabadi. Effects of sterol regulatory element-binding protein (SREBP) in chickens. Lipids in health and disease. 2012 Feb; 11(?):20. doi: 10.1186/1476-511x-11-20. [PMID: 22309629]
  • Jay S Kirkwood, Katie M Lebold, Cristobal L Miranda, Charlotte L Wright, Galen W Miller, Robert L Tanguay, Carrie L Barton, Maret G Traber, Jan F Stevens. Vitamin C deficiency activates the purine nucleotide cycle in zebrafish. The Journal of biological chemistry. 2012 Feb; 287(6):3833-41. doi: 10.1074/jbc.m111.316018. [PMID: 22170049]
  • Alain Hartmann, Aude Locatelli, Lucie Amoureux, Géraldine Depret, Claudy Jolivet, Eric Gueneau, Catherine Neuwirth. Occurrence of CTX-M Producing Escherichia coli in Soils, Cattle, and Farm Environment in France (Burgundy Region). Frontiers in microbiology. 2012; 3(?):83. doi: 10.3389/fmicb.2012.00083. [PMID: 22408639]
  • Agnieszka Jurecka, Elzbieta Jurkiewicz, Anna Tylki-Szymanska. Magnetic resonance imaging of the brain in adenylosuccinate lyase deficiency: a report of seven cases and a review of the literature. European journal of pediatrics. 2012 Jan; 171(1):131-8. doi: 10.1007/s00431-011-1503-9. [PMID: 21625931]
  • Osbaldo Resendis-Antonio, Magdalena Hernández, Emmanuel Salazar, Sandra Contreras, Gabriel Martínez Batallar, Yolanda Mora, Sergio Encarnación. Systems biology of bacterial nitrogen fixation: high-throughput technology and its integrative description with constraint-based modeling. BMC systems biology. 2011 Jul; 5(?):120. doi: 10.1186/1752-0509-5-120. [PMID: 21801415]
  • Giuseppe Lazzarino, Angela Maria Amorini, Valentina Di Pietro, Barbara Tavazzi. HPLC analysis for the clinical-biochemical diagnosis of inborn errors of metabolism of purines and pyrimidines. Methods in molecular biology (Clifton, N.J.). 2011; 708(?):99-117. doi: 10.1007/978-1-61737-985-7_5. [PMID: 21207285]
  • Barbara Roe, Elizabeth Kensicki, Robert Mohney, William W Hall. Metabolomic profile of hepatitis C virus-infected hepatocytes. PloS one. 2011; 6(8):e23641. doi: 10.1371/journal.pone.0023641. [PMID: 21853158]
  • Yu-chun Guo, Hui-long Xu, Fang-yu Chen, Sheng-yao Guo, Yi-yuan Liang, Kang-jing Liang, Wen-xiong Lin. [Differential protein analysis on the root response of rice with high phosphorous uptake efficiency to low phosphorous stress]. Ying yong sheng tai xue bao = The journal of applied ecology. 2010 Dec; 21(12):3231-8. doi: ". [PMID: 21443014]
  • Philippe Goffin, Bert van de Bunt, Marco Giovane, Johan H J Leveau, Sachie Höppener-Ogawa, Bas Teusink, Jeroen Hugenholtz. Understanding the physiology of Lactobacillus plantarum at zero growth. Molecular systems biology. 2010 Sep; 6(?):413. doi: 10.1038/msb.2010.67. [PMID: 20865006]
  • M Henneke, S Dreha-Kulaczewski, K Brockmann, M van der Graaf, M A A P Willemsen, U Engelke, P Dechent, A Heerschap, G Helms, R A Wevers, J Gärtner. In vivo proton MR spectroscopy findings specific for adenylosuccinate lyase deficiency. NMR in biomedicine. 2010 Jun; 23(5):441-5. doi: 10.1002/nbm.1480. [PMID: 20175147]
  • P S Chauhan, C S Nautiyal. The purB gene controls rhizosphere colonization by Pantoea agglomerans. Letters in applied microbiology. 2010 Feb; 50(2):205-10. doi: 10.1111/j.1472-765x.2009.02779.x. [PMID: 20002573]
  • Angela Sempere, Angela Arias, Guillermo Farré, Judith García-Villoria, Pilar Rodríguez-Pombo, Lurdes R Desviat, Begoña Merinero, Angels García-Cazorla, Maria A Vilaseca, Antonia Ribes, Rafael Artuch, Jaume Campistol. Study of inborn errors of metabolism in urine from patients with unexplained mental retardation. Journal of inherited metabolic disease. 2010 Feb; 33(1):1-7. doi: 10.1007/s10545-009-9004-y. [PMID: 20049533]
  • Carsten Henneges, Dino Bullinger, Richard Fux, Natascha Friese, Harald Seeger, Hans Neubauer, Stefan Laufer, Christoph H Gleiter, Matthias Schwab, Andreas Zell, Bernd Kammerer. Prediction of breast cancer by profiling of urinary RNA metabolites using Support Vector Machine-based feature selection. BMC cancer. 2009 Apr; 9(?):104. doi: 10.1186/1471-2407-9-104. [PMID: 19344524]
  • Martin Frenkel, Carsten Külheim, Hanna Johansson Jänkänpää, Oskar Skogström, Luca Dall'Osto, Jon Agren, Roberto Bassi, Thomas Moritz, Jon Moen, Stefan Jansson. Improper excess light energy dissipation in Arabidopsis results in a metabolic reprogramming. BMC plant biology. 2009 Jan; 9(?):12. doi: 10.1186/1471-2229-9-12. [PMID: 19171025]
  • Cyril Gitiaux, Irène Ceballos-Picot, Sandrine Marie, Vassili Valayannopoulos, Marlène Rio, Séverine Verrieres, Jean François Benoist, Marie Françoise Vincent, Isabelle Desguerre, Nadia Bahi-Buisson. Misleading behavioural phenotype with adenylosuccinate lyase deficiency. European journal of human genetics : EJHG. 2009 Jan; 17(1):133-6. doi: 10.1038/ejhg.2008.174. [PMID: 18830228]
  • Hanna Mierzewska, Bogna Schmidt-Sidor, Elzbieta Jurkiewicz, Anna Bogdańska, Katarzyna Kuśmierska, Tomasz Stepień. Severe encephalopathy with brain atrophy and hypomyelination due to adenylosuccinate lyase deficiency--MRI, clinical, biochemical and neuropathological findings of Polish patients. Folia neuropathologica. 2009; 47(4):314-20. doi: . [PMID: 20054783]
  • A Jurecka, A Tylki-Szymanska, M Zikanova, J Krijt, S Kmoch. D-ribose therapy in four Polish patients with adenylosuccinate lyase deficiency: absence of positive effect. Journal of inherited metabolic disease. 2008 Dec; 31 Suppl 2(?):S329-32. doi: 10.1007/s10545-008-0904-z. [PMID: 18649008]
  • Agnieszka Jurecka, Marie Zikanova, Anna Tylki-Szymanska, Jakub Krijt, Anna Bogdanska, Wanda Gradowska, Karolina Mullerova, Jolanta Sykut-Cegielska, Stanislav Kmoch, Ewa Pronicka. Clinical, biochemical and molecular findings in seven Polish patients with adenylosuccinate lyase deficiency. Molecular genetics and metabolism. 2008 Aug; 94(4):435-442. doi: 10.1016/j.ymgme.2008.04.013. [PMID: 18524658]
  • Wangzhen Guo, Caiping Cai, Changbiao Wang, Liang Zhao, Lei Wang, Tianzhen Zhang. A preliminary analysis of genome structure and composition in Gossypium hirsutum. BMC genomics. 2008 Jul; 9(?):314. doi: 10.1186/1471-2164-9-314. [PMID: 18590573]
  • Frances M Dupont. Metabolic pathways of the wheat (Triticum aestivum) endosperm amyloplast revealed by proteomics. BMC plant biology. 2008 Apr; 8(?):39. doi: 10.1186/1471-2229-8-39. [PMID: 18419817]
  • Petr Hornik, Petra Vyskocilová, David Friedecký, Anna Janostáková, Katerina Adamová, Tomás Adam. Analysis of aminoimidazole ribosides by capillary electrophoresis--diagnosing defects in second part of purine biosynthetic pathway. Clinica chimica acta; international journal of clinical chemistry. 2007 Feb; 376(1-2):184-9. doi: 10.1016/j.cca.2006.08.020. [PMID: 17010959]
  • Susen Hartmann, Jürgen G Okun, Christiane Schmidt, Claus-Dieter Langhans, Sven F Garbade, Peter Burgard, Dorothea Haas, Jörn Oliver Sass, William L Nyhan, Georg F Hoffmann. Comprehensive detection of disorders of purine and pyrimidine metabolism by HPLC with electrospray ionization tandem mass spectrometry. Clinical chemistry. 2006 Jun; 52(6):1127-37. doi: 10.1373/clinchem.2005.058842. [PMID: 16613999]
  • David L Scott, Gerold Diez, Wolfgang H Goldmann. Protein-lipid interactions: correlation of a predictive algorithm for lipid-binding sites with three-dimensional structural data. Theoretical biology & medical modelling. 2006 Mar; 3(?):17. doi: 10.1186/1742-4682-3-17. [PMID: 16569237]
  • Donna K Mahnke, Richard L Sabina. Calcium activates erythrocyte AMP deaminase [isoform E (AMPD3)] through a protein-protein interaction between calmodulin and the N-terminal domain of the AMPD3 polypeptide. Biochemistry. 2005 Apr; 44(14):5551-9. doi: 10.1021/bi048121p. [PMID: 15807549]
  • A M Marinaki, M Champion, M A Kurian, H A Simmonds, S Marie, M F Vincent, G van den Berghe, J A Duley, L D Fairbanks. Adenylosuccinate lyase deficiency--first British case. Nucleosides, nucleotides & nucleic acids. 2004 Oct; 23(8-9):1231-3. doi: 10.1081/ncn-200027494. [PMID: 15571235]
  • Patrick Edery, Stéphane Chabrier, Irène Ceballos-Picot, Sandrine Marie, Marie-Françoise Vincent, Marc Tardieu. Intrafamilial variability in the phenotypic expression of adenylosuccinate lyase deficiency: a report on three patients. American journal of medical genetics. Part A. 2003 Jul; 120A(2):185-90. doi: 10.1002/ajmg.a.20176. [PMID: 12833398]
  • M Castro, C Pérez-Cerdá, B Merinero, M J García, J Bernar, A Gil Nagel, J Torres, M Bermúdez, P Garavito, S Marie, F Vincent, G Van den Berghe, M Ugarte. Screening for adenylosuccinate lyase deficiency: clinical, biochemical and molecular findings in four patients. Neuropediatrics. 2002 Aug; 33(4):186-9. doi: 10.1055/s-2002-34493. [PMID: 12368987]
  • F Ciardo, C Salerno, P Curatolo. Neurologic aspects of adenylosuccinate lyase deficiency. Journal of child neurology. 2001 May; 16(5):301-8. doi: 10.1177/088307380101600501. [PMID: 11392513]
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