Homocitrulline (BioDeep_00000003674)

 

Secondary id: BioDeep_00000402859, BioDeep_00000405333

human metabolite PANOMIX_OTCML-2023 Endogenous blood metabolite


代谢物信息卡片


(2S)-2-amino-6-(carbamoylamino)hexanoic acid

化学式: C7H15N3O3 (189.111336)
中文名称: L-高瓜氨酸, 高瓜氨酸
谱图信息: 最多检出来源 Homo sapiens(blood) 0.09%

Reviewed

Last reviewed on 2024-06-29.

Cite this Page

Homocitrulline. BioDeep Database v3. PANOMIX ltd, a top metabolomics service provider from China. https://query.biodeep.cn/s/homocitrulline (retrieved 2024-09-17) (BioDeep RN: BioDeep_00000003674). Licensed under the Attribution-Noncommercial 4.0 International License (CC BY-NC 4.0).

分子结构信息

SMILES: C(CCNC(=O)N)CC(C(=O)O)N
InChI: InChI=1S/C7H15N3O3/c8-5(6(11)12)3-1-2-4-10-7(9)13/h5H,1-4,8H2,(H,11,12)(H3,9,10,13)/t5-/m0/s1

描述信息

Homocitrulline is a metabolite that can be detected in larger amounts in the urine of individuals with urea cycle disorders (OMIM 238970). The accumulation of carbamylphosphate due to depleted supply of ornithine for the urea cycle may be responsible for the enhanced synthesis of homocitrulline and homoarginine in some cases (PMID 2474087). Homocitrulline has been identified in the human placenta (PMID: 32033212).
Homocitrulline is a metabolite that can be detected in larger amounts in the urine of individuals with urea cycle disorders (OMIM 238970). The accumulation of carbamylphosphate due to depleted supply of ornithine for the urea cycle may be responsible for the enhanced synthesis of homocitrulline and homoarginine in some cases (PMID 2474087). [HMDB]
L-Homocitrulline is metabolized to homoarginine through homoargininosuccinate via the urea cycle pathway and its metabolic abnormality could lead to Lysinuric Protein Intolerance (LPI).
L-Homocitrulline is metabolized to homoarginine through homoargininosuccinate via the urea cycle pathway and its metabolic abnormality could lead to Lysinuric Protein Intolerance (LPI).

同义名列表

16 个代谢物同义名

(2S)-2-amino-6-(carbamoylamino)hexanoic acid; (S)-2-Amino-6-ureidohexanoic acid; N-ε-Carbamyl-L-lysine; 2-Amino-6-ureidohexanoic acid; N(6)-(Aminocarbonyl)-L-lysine; N6-(Aminocarbonyl)-L-lysine; N-epsilon-Carbamyl-L-lysine; N-Ε-carbamyl-L-lysine; N-e-Carbamyl-L-lysine; N6-Carbamoyl-L-lysine; N6-Carbamoyl-lysine; Ureidocaproic acid; Homo-L-citrulline; L-Homocitrulline; Homocitrulline; Homocitrulline



数据库引用编号

16 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)

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

  • Svetlana Baskal, Alexander Bollenbach, Dimitrios Tsikas. GC-MS Discrimination of Citrulline from Ornithine and Homocitrulline from Lysine by Chemical Derivatization: Evidence of Formation of N5-Carboxy-ornithine and N6-Carboxy-lysine. Molecules (Basel, Switzerland). 2021 Apr; 26(8):. doi: 10.3390/molecules26082301. [PMID: 33921162]
  • Emanuele De Simone, Lucia Di Micco, Gaetano La Manna, Biagio Di Iorio. [Protein carbamylation: what it is and why it concerns nephrologists]. Giornale italiano di nefrologia : organo ufficiale della Societa italiana di nefrologia. 2018 May; 35(3):. doi: NULL. [PMID: 29786184]
  • Stéphane Jaisson, Aurore Desmons, Manon Doué, Laëtitia Gorisse, Christine Pietrement, Philippe Gillery. Measurement of Homocitrulline, A Carbamylation-derived Product, in Serum and Tissues by LC-MS/MS. Current protocols in protein science. 2018 04; 92(1):e56. doi: 10.1002/cpps.56. [PMID: 30040196]
  • Michael S Lustgarten, Roger A Fielding. Metabolites related to renal function, immune activation, and carbamylation are associated with muscle composition in older adults. Experimental gerontology. 2017 12; 100(?):1-10. doi: 10.1016/j.exger.2017.10.003. [PMID: 29030163]
  • Morgan E Grams, Adrienne Tin, Casey M Rebholz, Tariq Shafi, Anna Köttgen, Ronald D Perrone, Mark J Sarnak, Lesley A Inker, Andrew S Levey, Josef Coresh. Metabolomic Alterations Associated with Cause of CKD. Clinical journal of the American Society of Nephrology : CJASN. 2017 Nov; 12(11):1787-1794. doi: 10.2215/cjn.02560317. [PMID: 28971980]
  • David A Bell, Sallie Elhayek, Ewa Cairns, Lillian Barra. Anti-homocitrullinated protein antibody isotype usage in rheumatoid arthritis and their unaffected first-degree relatives. Clinical and experimental rheumatology. 2017 Nov; 35(6):948-953. doi: NULL. [PMID: 28598783]
  • Yuki Taga, Keisuke Tanaka, Chieko Hamada, Masashi Kusubata, Kiyoko Ogawa-Goto, Shunji Hattori. Hydroxyhomocitrulline Is a Collagen-Specific Carbamylation Mark that Affects Cross-link Formation. Cell chemical biology. 2017 Oct; 24(10):1276-1284.e3. doi: 10.1016/j.chembiol.2017.08.010. [PMID: 28943354]
  • Jonathan D Jones, B JoNell Hamilton, William F C Rigby. Brief Report: Anti-Carbamylated Protein Antibodies in Rheumatoid Arthritis Patients Are Reactive With Specific Epitopes of the Human Fibrinogen β-Chain. Arthritis & rheumatology (Hoboken, N.J.). 2017 07; 69(7):1381-1386. doi: 10.1002/art.40098. [PMID: 28296337]
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  • Camille P Figueiredo, Holger Bang, Jayme Fogagnolo Cobra, Matthias Englbrecht, Axel J Hueber, Judith Haschka, Bernhard Manger, Arnd Kleyer, Michaela Reiser, Stephanie Finzel, Hans-Peter Tony, Stefan Kleinert, Joerg Wendler, Florian Schuch, Monika Ronneberger, Martin Feuchtenberger, Martin Fleck, Karin Manger, Wolfgang Ochs, Matthias Schmitt-Haendle, Hanns-Martin Lorenz, Hubert Nuesslein, Rieke Alten, Joerg Henes, Klaus Krueger, Jürgen Rech, Georg Schett. Antimodified protein antibody response pattern influences the risk for disease relapse in patients with rheumatoid arthritis tapering disease modifying antirheumatic drugs. Annals of the rheumatic diseases. 2017 Feb; 76(2):399-407. doi: 10.1136/annrheumdis-2016-209297. [PMID: 27323772]
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  • Aurore Desmons, Stéphane Jaisson, Christine Pietrement, Philippe Rieu, Alain Wynckel, Philippe Gillery. Homocitrulline: a new marker for differentiating acute from chronic renal failure. Clinical chemistry and laboratory medicine. 2016 Jan; 54(1):73-9. doi: 10.1515/cclm-2015-0398. [PMID: 26124058]
  • Elena B Lugli, Raquel E S M Correia, Roman Fischer, Karin Lundberg, Ken R Bracke, Anna B Montgomery, Benedikt M Kessler, Guy G Brusselle, Patrick J Venables. Expression of citrulline and homocitrulline residues in the lungs of non-smokers and smokers: implications for autoimmunity in rheumatoid arthritis. Arthritis research & therapy. 2015 Jan; 17(?):9. doi: 10.1186/s13075-015-0520-x. [PMID: 25600626]
  • Stéphane Jaisson, Mohsen Kerkeni, Izabella C R Santos-Weiss, Faouzi Addad, Mohammed Hammami, Philippe Gillery. Increased serum homocitrulline concentrations are associated with the severity of coronary artery disease. Clinical chemistry and laboratory medicine. 2015 Jan; 53(1):103-10. doi: 10.1515/cclm-2014-0642. [PMID: 25153409]
  • Dalia El-Gamal, Shailaja P Rao, Michael Holzer, Seth Hallström, Johannes Haybaeck, Martin Gauster, Christian Wadsack, Andrijana Kozina, Saša Frank, Rudolf Schicho, Rufina Schuligoi, Akos Heinemann, Gunther Marsche. The urea decomposition product cyanate promotes endothelial dysfunction. Kidney international. 2014 Nov; 86(5):923-31. doi: 10.1038/ki.2014.218. [PMID: 24940796]
  • Mathias Scinocca, David A Bell, Maud Racapé, Radha Joseph, Gary Shaw, John K McCormick, Dafna D Gladman, Janet Pope, Lillian Barra, Ewa Cairns. Antihomocitrullinated fibrinogen antibodies are specific to rheumatoid arthritis and frequently bind citrullinated proteins/peptides. The Journal of rheumatology. 2014 Feb; 41(2):270-9. doi: 10.3899/jrheum.130742. [PMID: 24429169]
  • Sanna Turunen, Marja-Kaisa Koivula, Jukka Melkko, Eeva Alasaarela, Petri Lehenkari, Juha Risteli. Different amounts of protein-bound citrulline and homocitrulline in foot joint tissues of a patient with anti-citrullinated protein antibody positive erosive rheumatoid arthritis. Journal of translational medicine. 2013 Sep; 11(?):224. doi: 10.1186/1479-5876-11-224. [PMID: 24060405]
  • Anasufiza Habib, Zabedah Md Yunus, Nor Azimah Azize, Gaik-Siew Ch'ng, Winnie Peitee Ong, Bee-Chin Chen, Ho-Torng Hsu, Ke-Juin Wong, James Pitt, Lock-Hock Ngu. Hyperexcretion of homocitrulline in a Malaysian patient with lysinuric protein intolerance. European journal of pediatrics. 2013 Sep; 172(9):1277-81. doi: 10.1007/s00431-013-1947-1. [PMID: 23358709]
  • Angela Zanatta, Carolina Maso Viegas, Anelise Miotti Tonin, Estela Natacha Brandt Busanello, Mateus Grings, Alana Pimentel Moura, Guilhian Leipnitz, Moacir Wajner. Disturbance of redox homeostasis by ornithine and homocitrulline in rat cerebellum: a possible mechanism of cerebellar dysfunction in HHH syndrome. Life sciences. 2013 Aug; 93(4):161-8. doi: 10.1016/j.lfs.2013.06.013. [PMID: 23806752]
  • W H Wilson Tang, Kevin Shrestha, Zeneng Wang, Allen G Borowski, Richard W Troughton, Allan L Klein, Stanley L Hazen. Protein carbamylation in chronic systolic heart failure: relationship with renal impairment and adverse long-term outcomes. Journal of cardiac failure. 2013 Apr; 19(4):219-24. doi: 10.1016/j.cardfail.2013.02.001. [PMID: 23582087]
  • Robert A Koeth, Kamyar Kalantar-Zadeh, Zeneng Wang, Xiaoming Fu, W H Wilson Tang, Stanley L Hazen. Protein carbamylation predicts mortality in ESRD. Journal of the American Society of Nephrology : JASN. 2013 Apr; 24(5):853-61. doi: 10.1681/asn.2012030254. [PMID: 23431074]
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