N6-succinyladenosine (BioDeep_00000019444)

   

human metabolite Endogenous blood metabolite


代谢物信息卡片


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

化学式: C14H17N5O8 (383.1077082)
中文名称: N6-琥珀酰腺苷
谱图信息: 最多检出来源 Homo sapiens(blood) 6.58%

Reviewed

Last reviewed on 2024-09-14.

Cite this Page

N6-succinyladenosine. BioDeep Database v3. PANOMIX ltd, a top metabolomics service provider from China. https://query.biodeep.cn/s/n6-succinyladenosine (retrieved 2024-09-19) (BioDeep RN: BioDeep_00000019444). Licensed under the Attribution-Noncommercial 4.0 International License (CC BY-NC 4.0).

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](CO)O1)O)O)C(=O)O
InChI: InChI=1S/C14H17N5O8/c20-2-6-9(23)10(24)13(27-6)19-4-17-8-11(15-3-16-12(8)19)18-5(14(25)26)1-7(21)22/h3-6,9-10,13,20,23-24H,1-2H2,(H,21,22)(H,25,26)(H,15,16,18)

描述信息

Succinyladenosine (SAdo) is one of the dephosphorylated enzyme substrate that accumulates in body fluids of patients with adenylosuccinate lyase (ADSL) deficiency, the other being 5-amino-4-imidazole-N-succinocarboxamide riboside (SAICAr). ADSL is an inherited metabolic disease characterized by various degrees of psychomotor retardation (PMID 15902552). The severity of the clinical presentation correlates with a low S-Ado/SAICAr ratio in body fluids (PMID: 15571235). Normally Succinyladenosine is not found in blood or CSF but may be detected in trace amounts in urine (OMIM 103050). Succinyladenosine is also found to be associated with fumarase deficiency, another inborn error of metabolism.
Succinyladenosine (SAdo) is one of the dephosphorylated enzyme substrate that accumulates in body fluids of patients with adenylosuccinate lyase (ADSL) deficiency, the other being 5-amino-4-imidazole-N-succinocarboxamide riboside (SAICAr). ADSL is an inherited metabolic disease characterized by various degrees of psychomotor retardation. (PMID 15902552)

同义名列表

20 个代谢物同义名

(2S)-2-({9-[(2R,3R,4S,5R)-3,4-dihydroxy-5-(hydroxymethyl)oxolan-2-yl]-9H-purin-6-yl}amino)butanedioic acid; N-(9-beta-delta-Ribofuranosyl-9H-purin-6-yl)-L-aspartic acid; 6-(1,2-Dicarboxyethylamino)-9-beta-delta-ribofuranosylpurine; 6-(1,2-Dicarboxyethylamino)-9-beta-D-ribofuranosylpurine; N-(9-beta-delta-Ribofuranosyl-9H-purin-6-yl)-L-aspartate; N-(9-beta-D-Ribofuranosyl-9H-purin-6-yl)-L-aspartic acid; 6-(1,2-Dicarboxyethylamino)-9-b-D-ribofuranosylpurine; N-(9-b-D-Ribofuranosyl-9H-purin-6-yl)-L-aspartic acid; 6-(1,2-Dicarboxyethylamino)-9-β-D-ribofuranosylpurine; N-(9-Β-D-ribofuranosyl-9H-purin-6-yl)-L-aspartic acid; N-(9-beta-D-Ribofuranosyl-9H-purin-6-yl)-L-aspartate; N-(9-Β-D-ribofuranosyl-9H-purin-6-yl)-L-aspartate; N-(9-b-D-Ribofuranosyl-9H-purin-6-yl)-L-aspartate; N-(9-Pentofuranosyl-9H-purin-6-yl)aspartic acid; N-9-Ribofuranosyl-9H-purin-6-yl-aspartic acid; N-9-Ribofuranosyl-9H-purin-6-yl-aspartate; (S)-N-(1,2-Dicarboxyethyl)-adenosine; atp - adenosine triphosphate; Succinyladenosine; Succinoadenosine



数据库引用编号

9 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

2 个相关的代谢反应过程信息。

Reactome(0)

BioCyc(0)

WikiPathways(2)

Plant Reactome(0)

INOH(0)

PlantCyc(0)

COVID-19 Disease Map(0)

PathBank(0)

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

  • Hanna-Riikka Heinonen, Miika Mehine, Netta Mäkinen, Annukka Pasanen, Esa Pitkänen, Auli Karhu, Nanna S Sarvilinna, Jari Sjöberg, Oskari Heikinheimo, Ralf Bützow, Lauri A Aaltonen, Eevi Kaasinen. Global metabolomic profiling of uterine leiomyomas. British journal of cancer. 2017 Dec; 117(12):1855-1864. doi: 10.1038/bjc.2017.361. [PMID: 29073636]
  • Toshihiro Kobayashi, Yuriko Matsumura, Toshihiko Ozawa, Hiroyuki Yanai, Atsuo Iwasawa, Toshiaki Kamachi, Kouichi Fujiwara, Noriaki Tanaka, Masahiro Kohno. Exploration of novel predictive markers in rat plasma of the early stages of chronic renal failure. Analytical and bioanalytical chemistry. 2014 Feb; 406(5):1365-76. doi: 10.1007/s00216-013-7472-2. [PMID: 24232639]
  • Jakub Krijt, Vaclava Skopova, Vaclava Adamkova, Renata Cermakova, Agnieszka Jurecka, Stanislav Kmoch, Marie Zikanova. The need for vigilance: false-negative screening for adenylosuccinate lyase deficiency caused by deribosylation of urinary biomarkers. Clinical biochemistry. 2013 Dec; 46(18):1899-901. doi: 10.1016/j.clinbiochem.2013.10.018. [PMID: 24183879]
  • Michiel A van Werkhoven, John A Duley, Ivan McGown, Teresa Munce, Jeremy L Freeman, James J Pitt. Early diagnosis of adenylosuccinate lyase deficiency using a high-throughput screening method and a trial of oral S-adenosyl-l-methionine as a treatment method. Developmental medicine and child neurology. 2013 Nov; 55(11):1060-4. doi: 10.1111/dmcn.12244. [PMID: 23937257]
  • Bee Chin Chen, Ivan N McGown, Meow Keong Thong, James Pitt, Zabedah M Yunus, Teck Beng Khoo, Lock Hock Ngu, John A Duley. Adenylosuccinate lyase deficiency in a Malaysian patient, with novel adenylosuccinate lyase gene mutations. Journal of inherited metabolic disease. 2010 Dec; 33 Suppl 3(?):S159-62. doi: 10.1007/s10545-010-9056-z. [PMID: 20177786]
  • 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]
  • 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]
  • 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]
  • V Race, S Marie, M F Vincent, G Van den Berghe. Clinical, biochemical and molecular genetic correlations in adenylosuccinate lyase deficiency. Human molecular genetics. 2000 Sep; 9(14):2159-65. doi: 10.1093/hmg/9.14.2159. [PMID: 10958654]
  • J Zeman, J Krijt, L Stratilová, H Hansíková, L Wenchich, S Kmoch, P Chrastina, J Houstek. Abnormalities in succinylpurines in fumarase deficiency: possible role in pathogenesis of CNS impairment. Journal of inherited metabolic disease. 2000 Jun; 23(4):371-4. doi: 10.1023/a:1005639516342. [PMID: 10896297]
  • F A van den Bergh, A N Bosschaart, G Hageman, M Duran, B Tien Poll-The. Adenylosuccinase deficiency with neonatal onset severe epileptic seizures and sudden death. Neuropediatrics. 1998 Feb; 29(1):51-3. doi: 10.1055/s-2007-973536. [PMID: 9553952]
  • S Sumi, K Kidouchi, S Ohba, Y Wada. Automated screening system for purine and pyrimidine metabolism disorders using high-performance liquid chromatography. Journal of chromatography. B, Biomedical applications. 1995 Oct; 672(2):233-9. doi: 10.1016/0378-4347(95)00228-b. [PMID: 8581129]
  • M Gross, B S Gathof, P Kölle, U Gresser. Capillary electrophoresis for screening of adenylosuccinate lyase deficiency. Electrophoresis. 1995 Oct; 16(10):1927-9. doi: 10.1002/elps.11501601318. [PMID: 8586067]
  • C Salerno, C Crifò. Microassay of adenylosuccinase by capillary electrophoresis. Analytical biochemistry. 1995 Apr; 226(2):377-9. doi: 10.1006/abio.1995.1240. [PMID: 7793642]
  • J Jaeken, F Van den Bergh, M F Vincent, P Casaer, G Van den Berghe. Adenylosuccinase deficiency: a newly recognized variant. Journal of inherited metabolic disease. 1992; 15(3):416-8. doi: 10.1007/bf02435992. [PMID: 1405483]
  • P K Laikind, J E Seegmiller, H E Gruber. Detection of 5'-phosphoribosyl-4-(N-succinylcarboxamide)-5-aminoimidazole in urine by use of the Bratton-Marshall reaction: identification of patients deficient in adenylosuccinate lyase activity. Analytical biochemistry. 1986 Jul; 156(1):81-90. doi: 10.1016/0003-2697(86)90158-2. [PMID: 3740420]
  • P K de Bree, S K Wadman, M Duran, H Fabery de Jonge. Diagnosis of inherited adenylosuccinase deficiency by thin-layer chromatography of urinary imidazoles and by automated cation exchange column chromatography of purines. Clinica chimica acta; international journal of clinical chemistry. 1986 May; 156(3):279-87. doi: 10.1016/0009-8981(86)90071-9. [PMID: 3719985]
  • S K Wadman, P K de Bree, M Duran, H F de Jonge. Detection of inherited adenylosuccinase deficiency by two dimensional thin layer chromatography of urinary imidazoles. Advances in experimental medicine and biology. 1986; 195 Pt A(?):21-5. doi: 10.1007/978-1-4684-5104-7_3. [PMID: 3728154]
  • S P Dutta, A Grossberg, A K Bhargava, G B Chheda. Radioimmunoassay for a novel urinary nucleoside, N6-succinyladenosine. Anticancer research. 1986 Jan; 6(1):135-8. doi: NULL. [PMID: 3954325]
  • J Jaeken, G Van den Berghe. An infantile autistic syndrome characterised by the presence of succinylpurines in body fluids. Lancet (London, England). 1984 Nov; 2(8411):1058-61. doi: . [PMID: 6150139]