Adenylsuccinic acid (BioDeep_00000003347)

 

Secondary id: BioDeep_00000175745

human metabolite Endogenous 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 (463.074)
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) 3.86%

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。

亚细胞结构定位 关联基因列表
Cytoplasm 12 ADSS1, ASL, DARS1, DMD, DNM2, EMP2, FH, HAND1, PML, PURA, SIVA1, UTRN
Peripheral membrane protein 4 DMD, DNM2, PML, UTRN
Endoplasmic reticulum membrane 1 PML
Nucleus 10 ADK, DMD, EMP2, FH, GATA4, HAND1, HAND2, PML, PURA, SIVA1
cytosol 10 ADK, ADSL, ADSS1, ASL, ASNS, DARS1, DMD, DNM2, FH, PML
dendrite 2 PURA, ROR2
nuclear body 1 GATA4
centrosome 1 DNM2
nucleoplasm 6 ADK, GATA4, HAND1, PML, SIVA1, UTRN
RNA polymerase II transcription regulator complex 2 GATA4, HAND1
Cell membrane 3 EMP2, ROR2, UTRN
Cytoplasmic side 3 DMD, PML, UTRN
Early endosome membrane 1 PML
Multi-pass membrane protein 1 EMP2
Golgi apparatus membrane 1 EMP2
Synapse 4 DARS1, DMD, DNM2, UTRN
cell junction 1 DNM2
cell surface 3 DMD, EMP2, ROR2
glutamatergic synapse 2 PURA, ROR2
Golgi apparatus 2 DNM2, EMP2
Golgi membrane 3 DNM2, EMP2, INS
neuromuscular junction 1 UTRN
neuronal cell body 2 PURA, ROR2
postsynapse 2 PURA, ROR2
sarcolemma 2 DMD, UTRN
Cytoplasm, cytosol 1 DARS1
Presynapse 1 DNM2
endosome 1 DNM2
plasma membrane 6 ADK, DMD, DNM2, EMP2, ROR2, UTRN
Membrane 6 ADSS1, DARS1, DMD, EMP2, ROR2, UTRN
apical plasma membrane 1 EMP2
extracellular exosome 5 ASL, DARS1, DNM2, FH, UTRN
extracellular space 1 INS
perinuclear region of cytoplasm 2 EMP2, INF2
mitochondrion 2 FH, SIVA1
protein-containing complex 4 ADSL, DMD, HAND2, UTRN
filopodium 2 DMD, UTRN
postsynaptic density 1 DNM2
Single-pass type I membrane protein 1 ROR2
Secreted 1 INS
extracellular region 1 INS
mitochondrial matrix 1 FH
transcription regulator complex 1 HAND2
Cytoplasm, cytoskeleton, microtubule organizing center, centrosome 1 DNM2
nuclear membrane 1 PML
Z disc 1 DMD
cytoplasmic vesicle 1 EMP2
nucleolus 2 HAND1, PML
midbody 1 DNM2
Membrane, clathrin-coated pit 1 DNM2
apical part of cell 1 EMP2
clathrin-coated pit 1 DNM2
recycling endosome 1 DNM2
postsynaptic membrane 3 DMD, DNM2, UTRN
Apical cell membrane 1 EMP2
Cell membrane, sarcolemma 1 DMD
Cytoplasm, perinuclear region 2 EMP2, INF2
Membrane raft 2 DMD, EMP2
Cytoplasm, cytoskeleton 2 DMD, DNM2
focal adhesion 1 DNM2
microtubule 2 DNM2, ROR2
Nucleus, PML body 1 PML
PML body 1 PML
Nucleus outer membrane 2 DMD, UTRN
nuclear outer membrane 2 DMD, UTRN
Postsynaptic cell membrane 1 DMD
receptor complex 1 ROR2
neuron projection 1 DNM2
chromatin 3 GATA4, HAND1, HAND2
Cytoplasmic vesicle, phagosome membrane 1 DNM2
Cell projection, phagocytic cup 1 DNM2
phagocytic cup 1 DNM2
phagocytic vesicle membrane 1 DNM2
Chromosome 1 FH
cytoskeleton 2 DMD, UTRN
Cell projection, podosome 1 DNM2
podosome 1 DNM2
Cytoplasm, cytoskeleton, microtubule organizing center, centrosome, centriole 1 DNM2
centriole 1 DNM2
Nucleus, nucleolus 1 HAND1
chromosome, telomeric region 2 PML, PURA
actin filament 1 INF2
site of double-strand break 1 FH
endosome lumen 1 INS
Nucleus, nucleoplasm 1 HAND1
filopodium membrane 2 DMD, UTRN
clathrin-coated vesicle 1 DNM2
secretory granule lumen 1 INS
Golgi lumen 1 INS
endoplasmic reticulum lumen 1 INS
nuclear matrix 1 PML
endocytic vesicle membrane 1 DNM2
Cytoplasmic vesicle, clathrin-coated vesicle 1 DNM2
transport vesicle 1 INS
Endoplasmic reticulum-Golgi intermediate compartment membrane 1 INS
Single-pass type IV membrane protein 2 DMD, UTRN
[Isoform 2]: Cytoplasm 1 ADK
clathrin-coated endocytic vesicle membrane 1 ROR2
uropod 1 DNM2
[Isoform 1]: Nucleus 1 ADK
costamere 1 DMD
Synapse, synaptosome 1 DNM2
aminoacyl-tRNA synthetase multienzyme complex 1 DARS1
dystrophin-associated glycoprotein complex 2 DMD, UTRN
Cell projection, uropodium 1 DNM2
cell-substrate junction 1 DMD
[Isoform Mitochondrial]: Mitochondrion 1 FH
[Isoform Cytoplasmic]: Cytoplasm, cytosol 1 FH
neuron projection terminus 1 DMD
syntrophin complex 1 DMD
organelle 1 DMD
plasma membrane bounded cell projection 1 DMD


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]
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  • 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]
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  • 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]
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  • 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]
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