L-Homocitrulline (BioDeep_00000402859)
Main id: BioDeep_00000003674
natural product PANOMIX_OTCML-2023
代谢物信息卡片
L-Homocitrulline
化学式: C7H15N3O3 (189.1113)
中文名称: L-高瓜氨酸, l-高瓜氨酸
谱图信息:
最多检出来源 () 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
A L-lysine derivative that is L-lysine having a carbamoyl group at the N(6)-position. It is found in individuals with urea cycle disorders.
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
3 个代谢物同义名
数据库引用编号
11 个数据库交叉引用编号
- ChEBI: CHEBI:17443
- KEGG: C02427
- PubChem: 65072
- CAS: 1190-49-4
- MoNA: PM000954
- MetaboLights: MTBLC17443
- PubChem: 5455
- PDB-CCD: YHA
- NIKKAJI: J14.380D
- medchemexpress: HY-W018004
- KNApSAcK: 17443
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)
1 个相关的物种来源信息
- 569774 - 金线莲: -
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] - Veronika Binder, Brith Bergum, Stéphane Jaisson, Philippe Gillery, Carsten Scavenius, Endy Spriet, Anne Karin Nyhaug, Helen M Roberts, Iain L C Chapple, Annelie Hellvard, Nicolas Delaleu, Piotr Mydel. Impact of fibrinogen carbamylation on fibrin clot formation and stability.
Thrombosis and haemostasis.
2017 05; 117(5):899-910. doi:
10.1160/th16-09-0704. [PMID: 28382370] - 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] - Jia Teng Sun, Ke Yang, Jing Yan Mao, Wei Feng Shen, Lin Lu, Qi Hong Wu, Yan Ping Wang, Li Ping Wu, Rui Yan Zhang. Cyanate-Impaired Angiogenesis: Association With Poor Coronary Collateral Growth in Patients With Stable Angina and Chronic Total Occlusion.
Journal of the American Heart Association.
2016 12; 5(12):. doi:
10.1161/jaha.116.004700. [PMID: 27986757] - Sanna Turunen, Marja-Kaisa Koivula, Leila Risteli, Juha Risteli. Ureido group-specific antibodies are induced in rabbits immunized with citrulline- or homocitrulline-containing antigens.
Autoimmunity.
2016 11; 49(7):459-465. doi:
10.3109/08916934.2016.1171853. [PMID: 27098309] - Zeneng Wang, Joseph A DiDonato, Jennifer Buffa, Suzy A Comhair, Mark A Aronica, Raed A Dweik, Nancy A Lee, James J Lee, Mary Jane Thomassen, Mani Kavuru, Serpil C Erzurum, Stanley L Hazen. Eosinophil Peroxidase Catalyzed Protein Carbamylation Participates in Asthma.
The Journal of biological chemistry.
2016 Oct; 291(42):22118-22135. doi:
10.1074/jbc.m116.750034. [PMID: 27587397] - Stéphane Jaisson, Isabelle Kazes, Aurore Desmons, Fouad Fadel, Jean-Baptiste Oudart, Izabella C R Dos Santos-Weiss, Hervé Millart, Fatouma Touré, Philippe Rieu, Philippe Gillery. Homocitrulline as marker of protein carbamylation in hemodialyzed patients.
Clinica chimica acta; international journal of clinical chemistry.
2016 Sep; 460(?):5-10. doi:
10.1016/j.cca.2016.06.009. [PMID: 27302313] - H Koppejan, L A Trouw, J Sokolove, L J Lahey, T J W Huizinga, I A Smolik, D B Robinson, H S El-Gabalawy, R E M Toes, C A Hitchon. Role of Anti-Carbamylated Protein Antibodies Compared to Anti-Citrullinated Protein Antibodies in Indigenous North Americans With Rheumatoid Arthritis, Their First-Degree Relatives, and Healthy Controls.
Arthritis & rheumatology (Hoboken, N.J.).
2016 09; 68(9):2090-8. doi:
10.1002/art.39664. [PMID: 26946484] - 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] - Carolina Maso Viegas, Estela Natacha Brandt Busanello, Anelise Miotti Tonin, Mateus Grings, Alana Pimentel Moura, Luciana Ritter, Angela Zanatta, Lisiane Aurélio Knebel, Vannessa Araujo Lobato, Letícia Ferreira Pettenuzzo, Carmen Regla Vargas, Guilhian Leipnitz, Moacir Wajner. Chronic postnatal ornithine administration to rats provokes learning deficit in the open field task.
Metabolic brain disease.
2012 Dec; 27(4):479-86. doi:
10.1007/s11011-012-9322-x. [PMID: 22699997] - Carolina Maso Viegas, Anelise Miotti Tonin, Angela Zanatta, Bianca Seminotti, Estela Natacha Brandt Busanello, Carolina Gonçalves Fernandes, Alana Pimentel Moura, Guilhian Leipnitz, Moacir Wajner. Impairment of brain redox homeostasis caused by the major metabolites accumulating in hyperornithinemia-hyperammonemia-homocitrullinuria syndrome in vivo.
Metabolic brain disease.
2012 Dec; 27(4):521-30. doi:
10.1007/s11011-012-9327-5. [PMID: 22798168] - Cédric Delporte, Thierry Franck, Caroline Noyon, Damien Dufour, Alexandre Rousseau, Philippe Madhoun, Jean-Marc Desmet, Didier Serteyn, Martine Raes, Joëlle Nortier, Michel Vanhaeverbeek, Nicole Moguilevsky, Jean Nève, Luc Vanhamme, Pierre Van Antwerpen, Karim Zouaoui Boudjeltia. Simultaneous measurement of protein-bound 3-chlorotyrosine and homocitrulline by LC-MS/MS after hydrolysis assisted by microwave: application to the study of myeloperoxidase activity during hemodialysis.
Talanta.
2012 Sep; 99(?):603-9. doi:
10.1016/j.talanta.2012.06.044. [PMID: 22967600] - Stéphane Jaisson, Laëtitia Gorisse, Christine Pietrement, Philippe Gillery. Quantification of plasma homocitrulline using hydrophilic interaction liquid chromatography (HILIC) coupled to tandem mass spectrometry.
Analytical and bioanalytical chemistry.
2012 Feb; 402(4):1635-41. doi:
10.1007/s00216-011-5619-6. [PMID: 22160237] - Olajumoke O Oladipo, Annette L Weindel, Al N Saunders, Dennis J Dietzen. Impact of premature birth and critical illness on neonatal range of plasma amino acid concentrations determined by LC-MS/MS.
Molecular genetics and metabolism.
2011 Dec; 104(4):476-9. doi:
10.1016/j.ymgme.2011.08.020. [PMID: 21908220] - Carolina Maso Viegas, Estela Natacha Brandt Busanello, Anelise Miotti Tonin, Alana Pimentel de Moura, Mateus Grings, Luciana Ritter, Patrícia Fernanda Schuck, Gustavo da Costa Ferreira, Angela Sitta, Carmen Regla Vargas, Moacir Wajner. Dual mechanism of brain damage induced in vivo by the major metabolites accumulating in hyperornithinemia-hyperammonemia-homocitrullinuria syndrome.
Brain research.
2011 Jan; 1369(?):235-44. doi:
10.1016/j.brainres.2010.10.112. [PMID: 21059345] - Hongxia Zhang, Lina Ding, Xuemei Fang, Zhimin Shi, Yating Zhang, Hebing Chen, Xianzhong Yan, Jiayin Dai. Biological responses to perfluorododecanoic acid exposure in rat kidneys as determined by integrated proteomic and metabonomic studies.
PloS one.
2011; 6(6):e20862. doi:
10.1371/journal.pone.0020862. [PMID: 21677784] - Mohammad Ali Ghaffari, Mehrnoosh Shanaki. In vitro inhibition of low density lipoprotein carbamylation by vitamins, as an ameliorating atherosclerotic risk in uremic patients.
Scandinavian journal of clinical and laboratory investigation.
2010 Apr; 70(2):122-7. doi:
10.3109/00365511003624137. [PMID: 20156036] - Alexandre Umpierrez Amaral, Guilhian Leipnitz, Carolina Gonçalves Fernandes, Bianca Seminotti, Angela Zanatta, Carolina Maso Viegas, Carlos Severo Dutra-Filho, Moacir Wajner. Evidence that the major metabolites accumulating in hyperornithinemia-hyperammonemia-homocitrullinuria syndrome induce oxidative stress in brain of young rats.
International journal of developmental neuroscience : the official journal of the International Society for Developmental Neuroscience.
2009 Nov; 27(7):635-41. doi:
10.1016/j.ijdevneu.2009.08.004. [PMID: 19683047] - Chiara Morini, Paolo Capozzi, Sara Boenzi, Cristiano Rizzo, Filippo Maria Santorelli, Carlo Dionisi-Vici. Retinal degeneration.
Ophthalmology.
2009 Aug; 116(8):1593, 1593.e1. doi:
10.1016/j.ophtha.2009.03.039. [PMID: 19651321] - Alessandra Tessa, Giuseppe Fiermonte, Carlo Dionisi-Vici, Eleonora Paradies, Matthias R Baumgartner, Yin-Hsiu Chien, Carmela Loguercio, Helene Ogier de Baulny, Marie-Cecile Nassogne, Manuel Schiff, Federica Deodato, Giancarlo Parenti, S Lane Rutledge, M Antonia Vilaseca, Mariarosa A B Melone, Gioacchino Scarano, Luiz Aldamiz-Echevarría, Guy Besley, John Walter, Eugenia Martinez-Hernandez, Jose M Hernandez, Ciro L Pierri, Ferdinando Palmieri, Filippo M Santorelli. Identification of novel mutations in the SLC25A15 gene in hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome: a clinical, molecular, and functional study.
Human mutation.
2009 May; 30(5):741-8. doi:
10.1002/humu.20930. [PMID: 19242930] - F-G Debray, M Lambert, B Lemieux, J F Soucy, R Drouin, D Fenyves, J Dubé, B Maranda, R Laframboise, G A Mitchell. Phenotypic variability among patients with hyperornithinaemia-hyperammonaemia-homocitrullinuria syndrome homozygous for the delF188 mutation in SLC25A15.
Journal of medical genetics.
2008 Nov; 45(11):759-64. doi:
10.1136/jmg.2008.059097. [PMID: 18978333] - Wolfgang Jelkmann. 'O', erythropoietin carbamoylation versus carbamylation.
Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association.
2008 Sep; 23(9):3033; author reply 3033-4. doi:
10.1093/ndt/gfn342. [PMID: 18556745] - A A Mhanni, A Chan, M Collison, B Seifert, D C Lehotay, Ah Sokoro, H Q Huynh, C R Greenberg. Hyperornithinemia-hyperammonemia-homocitrullinuria syndrome (HHH) presenting with acute fulminant hepatic failure.
Journal of pediatric gastroenterology and nutrition.
2008 Mar; 46(3):312-5. doi:
10.1097/mpg.0b013e318145a8e5. [PMID: 18376250] - Zeneng Wang, Stephen J Nicholls, E Rene Rodriguez, Outi Kummu, Sohvi Hörkkö, John Barnard, Wanda F Reynolds, Eric J Topol, Joseph A DiDonato, Stanley L Hazen. Protein carbamylation links inflammation, smoking, uremia and atherogenesis.
Nature medicine.
2007 Oct; 13(10):1176-84. doi:
10.1038/nm1637. [PMID: 17828273] - Osama Y Al-Dirbashi, Zuhair N Al-Hassnan, Mohamed S Rashed. Determination of homocitrulline in urine of patients with HHH syndrome by liquid chromatography tandem mass spectrometry.
Analytical and bioanalytical chemistry.
2006 Dec; 386(7-8):2013-7. doi:
10.1007/s00216-006-0831-5. [PMID: 17053917] - S Fecarotta, G Parenti, P Vajro, A Zuppaldi, R Della Casa, M T Carbone, A Correra, G Torre, S Riva, C Dionisi-Vici, F M Santorelli, G Andria. HHH syndrome (hyperornithinaemia, hyperammonaemia, homocitrullinuria), with fulminant hepatitis-like presentation.
Journal of inherited metabolic disease.
2006 Feb; 29(1):186-9. doi:
10.1007/s10545-006-0120-7. [PMID: 16601889] - Ercan Ok, Alexei G Basnakian, Eugene O Apostolov, Yousri M Barri, Sudhir V Shah. Carbamylated low-density lipoprotein induces death of endothelial cells: a link to atherosclerosis in patients with kidney disease.
Kidney international.
2005 Jul; 68(1):173-8. doi:
10.1111/j.1523-1755.2005.00391.x. [PMID: 15954906] - Stanley H Korman, Naomi Kanazawa, Bassam Abu-Libdeh, Alisa Gutman, Seiichi Tsujino. Hyperornithinemia, hyperammonemia, and homocitrullinuria syndrome with evidence of mitochondrial dysfunction due to a novel SLC25A15 (ORNT1) gene mutation in a Palestinian family.
Journal of the neurological sciences.
2004 Mar; 218(1-2):53-8. doi:
10.1016/j.jns.2003.10.017. [PMID: 14759633] - Giuseppe Fiermonte, Vincenza Dolce, Laura David, Filippo Maria Santorelli, Carlo Dionisi-Vici, Ferdinando Palmieri, John E Walker. The mitochondrial ornithine transporter. Bacterial expression, reconstitution, functional characterization, and tissue distribution of two human isoforms.
The Journal of biological chemistry.
2003 Aug; 278(35):32778-83. doi:
10.1074/jbc.m302317200. [PMID: 12807890] - José A Camacho, Natalia Rioseco-Camacho, Dario Andrade, John Porter, Jin Kong. Cloning and characterization of human ORNT2: a second mitochondrial ornithine transporter that can rescue a defective ORNT1 in patients with the hyperornithinemia-hyperammonemia-homocitrullinuria syndrome, a urea cycle disorder.
Molecular genetics and metabolism.
2003 Aug; 79(4):257-71. doi:
10.1016/s1096-7192(03)00105-7. [PMID: 12948741] - Seiichi Tsujino, Takeshi Miyamoto, Naomi Kanazawa. [Mitochondrial ornithine transporter deficiency].
Nihon rinsho. Japanese journal of clinical medicine.
2002 Apr; 60 Suppl 4(?):779-82. doi:
NULL. [PMID: 12013998] - A C Gallagher, M Pike, S Standing. HHH syndrome associated with callosal agenesis and disordered neuronal migration.
Developmental medicine and child neurology.
2001 Jun; 43(6):430-1. doi:
10.1111/j.1469-8749.2001.tb00237.x. [PMID: 11409836] - L M Kraus, L Gaber, C R Handorf, H P Marti, A P Kraus. Carbamoylation of glomerular and tubular proteins in patients with kidney failure: a potential mechanism of ongoing renal damage.
Swiss medical weekly.
2001 Mar; 131(11-12):139-4. doi:
10.4414/smw.2001.09691. [PMID: 11416886] - J Mühling, M G Dehne, M Fuchs, A Sablotzki, S Weiss, J Spatz, G Hempelmann. Conscientious metabolic monitoring on a patient with hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome undergoing anaesthesia.
Amino acids.
2001; 21(3):303-18. doi:
10.1007/s007260170016. [PMID: 11764411] - T Ohura, O Sakamoto. [Hyperornithinemia-hyperammonemia-homocitrullinuria syndrome].
Ryoikibetsu shokogun shirizu.
2001; ?(33):835-7. doi:
NULL. [PMID: 11462710] - C Salerno, P D'Eufemia, M Celli, R Finocchiaro, C Crifò, O Giardini. Determination of urinary orotic acid and uracil by capillary zone electrophoresis.
Journal of chromatography. B, Biomedical sciences and applications.
1999 Oct; 734(1):175-8. doi:
10.1016/s0378-4347(99)00337-0. [PMID: 10574204] - E Zammarchi, F Ciani, E Pasquini, G Buonocore, V E Shih, M A Donati, G Bonocore. Neonatal onset of hyperornithinemia-hyperammonemia-homocitrullinuria syndrome with favorable outcome.
The Journal of pediatrics.
1997 Sep; 131(3):440-3. doi:
10.1016/s0022-3476(97)80072-8. [PMID: 9329423] - E Zammarchi, M A Donati, L Filippi, M Resti. Cryptogenic hepatitis masking the diagnosis of ornithine transcarbamylase deficiency.
Journal of pediatric gastroenterology and nutrition.
1996 May; 22(4):380-3. doi:
10.1097/00005176-199605000-00007. [PMID: 8732901] - R G Gray, A Green, S Hall, C McKeown. Prenatal exclusion of the HHH syndrome.
Prenatal diagnosis.
1995 May; 15(5):474-6. doi:
10.1002/pd.1970150511. [PMID: 7644438] - L M Kraus, A J Elberger, C R Handorf, M J Pabst, A P Kraus. Urea-derived cyanate forms epsilon-amino-carbamoyl-lysine (homocitrulline) in leukocyte proteins in patients with end-stage renal disease on peritoneal dialysis.
The Journal of laboratory and clinical medicine.
1994 Jun; 123(6):882-91. doi:
. [PMID: 8201267] - L Smith, M A Lambert, P Brochu, G Jasmin, I A Qureshi, E G Seidman. Hyperornithinemia, hyperammonemia, homocitrullinuria (HHH) syndrome: presentation as acute liver disease with coagulopathy.
Journal of pediatric gastroenterology and nutrition.
1992 Nov; 15(4):431-6. doi:
10.1097/00005176-199211000-00011. [PMID: 1469525] - J F Lemay, M A Lambert, G A Mitchell, M Vanasse, D Valle, J F Arbour, J Dubé, J Flessas, M Laberge, L Lafleur. Hyperammonemia-hyperornithinemia-homocitrullinuria syndrome: neurologic, ophthalmologic, and neuropsychologic examination of six patients.
The Journal of pediatrics.
1992 Nov; 121(5 Pt 1):725-30. doi:
10.1016/s0022-3476(05)81900-6. [PMID: 1432421] - V E Shih, R Laframboise, R Mandell, J Pichette. Neonatal form of the hyperornithinaemia, hyperammonaemia, and homocitrullinuria (HHH) syndrome and prenatal diagnosis.
Prenatal diagnosis.
1992 Sep; 12(9):717-23. doi:
10.1002/pd.1970120905. [PMID: 1438066] - H Shimizu. [The dibasic amino acid metabolic disorders].
Nihon rinsho. Japanese journal of clinical medicine.
1992 Jul; 50(7):1555-60. doi:
NULL. [PMID: 1404883] - H Shigeto, T Yamada, T Kobayashi, I Goto. [A case of hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome with spastic paraparesis and severe distal muscle atrophy of lower limbs].
Rinsho shinkeigaku = Clinical neurology.
1992 Jul; 32(7):729-32. doi:
NULL. [PMID: 1291165] - A B Burlina, V Ferrari, C Dionisi-Vici, A Bordugo, F Zacchello, M Tuchman. Allopurinol challenge test in children.
Journal of inherited metabolic disease.
1992; 15(5):707-12. doi:
10.1007/bf01800010. [PMID: 1434508] - S Tsujino, T Suzuki, T Azuma, S Higa, S Sakoda, S Kishimoto. Hyperornithinemia, hyperammonemia and homocitrullinuria--a case report and study of ornithine metabolism using in vivo deuterium labelling.
Clinica chimica acta; international journal of clinical chemistry.
1991 Sep; 201(1-2):129-33. doi:
10.1016/0009-8981(91)90034-a. [PMID: 1790616] - L M Kraus, S Miyamura, B R Pecha, A P Kraus. Carbamoylation of hemoglobin in uremic patients determined by antibody specific for homocitrulline (carbamoylated epsilon-N-lysine).
Molecular immunology.
1991 Apr; 28(4-5):459-63. doi:
10.1016/0161-5890(91)90159-h. [PMID: 2062323] - I Koshiishi, Y Kobori, T Imanari. Determination of citrulline and homocitrulline by high-performance liquid chromatography with post-column derivatization.
Journal of chromatography.
1990 Oct; 532(1):37-43. doi:
10.1016/s0378-4347(00)83749-4. [PMID: 2079537] - M Tuchman, D S Knopman, V E Shih. Episodic hyperammonemia in adult siblings with hyperornithinemia, hyperammonemia, and homocitrullinuria syndrome.
Archives of neurology.
1990 Oct; 47(10):1134-7. doi:
10.1001/archneur.1990.00530100104022. [PMID: 2222247] - I Koshiishi, T Imanari. State analysis of endogenous cyanate ion in human plasma.
Journal of pharmacobio-dynamics.
1990 Apr; 13(4):254-8. doi:
10.1248/bpb1978.13.254. [PMID: 2117063] - B Chadefaux, J P Bonnefont, D Rabier, V E Shih, J M Saudubray, P Kamoun. Potential for the prenatal diagnosis of hyperornithinemia, hyperammonemia, and homocitrullinuria syndrome.
American journal of medical genetics.
1989 Feb; 32(2):264. doi:
10.1002/ajmg.1320320228. [PMID: 2929667] - T Kato, M Sano, N Mizutani. Homocitrullinuria and homoargininuria in lysinuric protein intolerance.
Journal of inherited metabolic disease.
1989; 12(2):157-61. doi:
10.1007/bf01800719. [PMID: 2474087] - J Botschner, D W Smith, O Simell, C R Scriver. Comparison of ornithine metabolism in hyperornithinaemia-hyperammonaemia-homocitrullinuria syndrome, lysinuric protein intolerance and gyrate atrophy fibroblasts.
Journal of inherited metabolic disease.
1989; 12(1):33-40. doi:
10.1007/bf01805528. [PMID: 2501580] - K Oyanagi. [Hyperornithinemia, hyperammonemia and homocitrullinuria due to a defect of mitochondrial membrane transport].
Tanpakushitsu kakusan koso. Protein, nucleic acid, enzyme.
1988 Apr; 33(5):843-6. doi:
NULL. [PMID: 3270901] - I Inoue, T Saheki, K Kayanuma, M Uono, M Nakajima, K Takeshita, R Koike, T Yuasa, T Miyatake, K Sakoda. Biochemical analysis of decreased ornithine transport activity in the liver mitochondria from patients with hyperornithinemia, hyperammonemia and homocitrullinuria.
Biochimica et biophysica acta.
1988 Jan; 964(1):90-5. doi:
10.1016/0304-4165(88)90071-2. [PMID: 3334877] - T Kato, M Sano, N Mizutani, C Hayakawa. Homocitrullinuria and homoargininuria in hyperargininaemia.
Journal of inherited metabolic disease.
1988; 11(3):261-5. doi:
10.1007/bf01800367. [PMID: 2466169] - R Noguchi, Y Tomita, M Hashiba, K Shimoji. [Anesthesia for a patient with the hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome].
Masui. The Japanese journal of anesthesiology.
1988 Jan; 37(1):86-92. doi:
NULL. [PMID: 3367506] - M Nakajima, S Ishii, T Mito, K Takeshita, S Takashima, H Takakura, I Inoue, T Saheki, H Akiyoshi, K Ichihara. Clinical, biochemical and ultrastructural study on the pathogenesis of hyperornithinemia-hyperammonemia-homocitrullinuria syndrome.
Brain & development.
1988; 10(3):181-5. doi:
10.1016/s0387-7604(88)80025-1. [PMID: 3407856] - C Dionisi Vici, C Bachmann, M Gambarara, J P Colombo, G Sabetta. Hyperornithinemia-hyperammonemia-homocitrullinuria syndrome: low creatine excretion and effect of citrulline, arginine, or ornithine supplement.
Pediatric research.
1987 Sep; 22(3):364-7. doi:
10.1203/00006450-198709000-00025. [PMID: 3116497] - B A Gordon, D P Gatfield, M D Haust. The hyperornithinemia, hyperammonemia, homocitrullinuria syndrome: an ornithine transport defect remediable with ornithine supplements.
Clinical and investigative medicine. Medecine clinique et experimentale.
1987 Jul; 10(4):329-36. doi:
. [PMID: 3652557] - R Koike, K Fujimori, T Yuasa, T Miyatake. [Hyperornithinemia, hyperammonemia and homocitrullinuria syndrome in a family].
Rinsho shinkeigaku = Clinical neurology.
1987 Apr; 27(4):465-71. doi:
NULL. [PMID: 3621746] - T Kato, M Sano, N Mizutani. Inhibitory effect of intravenous lysine infusion on urea cycle metabolism.
European journal of pediatrics.
1987 Jan; 146(1):56-8. doi:
10.1007/bf00647285. [PMID: 3107993] - M Rodes, A Ribes, M Pineda, L Alvarez, I Fabregas, E Fernandez Alvarez, F X Coude, G Grimber. A new family affected by the syndrome of hyperornithinaemia, hyperammonaemia and homocitrullinuria.
Journal of inherited metabolic disease.
1987; 10(1):73-81. doi:
10.1007/bf01799492. [PMID: 3106719] - G Michaelis, J Biscoping, G Hempelmann. [H-H-H syndrome: a rare disease also relevant in anesthesiology].
Anasthesie, Intensivtherapie, Notfallmedizin.
1986 Dec; 21(6):315-7. doi:
NULL. [PMID: 3812931] - F A Hommes, R A Roesel, K Metoki, P L Hartlage, P R Dyken. Studies on a case of HHH-syndrome (hyperammonemia, hyperornithinemia, homocitrullinuria).
Neuropediatrics.
1986 Feb; 17(1):48-52. doi:
10.1055/s-2008-1052499. [PMID: 3960284] - L R Gjessing, H A Lunde, T Undrum, H Broch, A Alme, S O Lie. A new patient with hyperornithinaemia, hyperammonaemia and homocitrullinuria treated early with low protein diet.
Journal of inherited metabolic disease.
1986; 9(2):186-92. doi:
10.1007/bf01799457. [PMID: 3091924] - O Simell, S Mackenzie, C L Clow, C R Scriver. Ornithine loading did not prevent induced hyperammonemia in a patient with hyperornithinemia-hyperammonemia-homocitrullinuria syndrome.
Pediatric research.
1985 Dec; 19(12):1283-7. doi:
10.1203/00006450-198512000-00016. [PMID: 4080446] - F A Hommes, A G Eller, B A Evans, A L Carter. Reconstitution of ornithine transport in liposomes with Lubrol extracts of mitochondria.
FEBS letters.
1984 May; 170(1):131-4. doi:
10.1016/0014-5793(84)81384-8. [PMID: 6723958] - A L Carter, A G Eller, S Rufo, K Metoki, F A Hommes. Further evidence for a separate enzymic entity for the synthesis of homocitrulline, distinct from the regular ornithine transcarbamylase.
Enzyme.
1984; 32(1):26-36. doi:
10.1159/000469447. [PMID: 6434302] - K Metoki, F A Hommes, P Dyken, C Kelloes, J Trefz. Ultrastructural changes in fibroblast mitochondria of a patient with HHH-syndrome.
Journal of inherited metabolic disease.
1984; 7(4):147-50. doi:
10.1007/bf01805595. [PMID: 6441860] - K Metoki, F A Hommes. The pH of mitochondria of fibroblasts from a hyperornithinaemia, hyperammonaemia, homocitrullinuria-syndrome patient.
Journal of inherited metabolic disease.
1984; 7(1):9-11. doi:
10.1007/bf01805611. [PMID: 6429444] - J Rajantie, O Simell, J Perheentupa. Oral administration of epsilon N-acetyllysine and homocitrulline in lysinuric protein intolerance.
The Journal of pediatrics.
1983 Mar; 102(3):388-90. doi:
10.1016/s0022-3476(83)80654-4. [PMID: 6402575] - K Oyanagi, A Tsuchiyama, Y Itakura, H Sogawa, K Wagatsuma, T Nakao, S Sakamoto, A Yachi. The mechanism of hyperammonaemia and hyperornithinaemia in the syndrome of hyperornithinaemia, hyperammonaemia with homocitrullinuria.
Journal of inherited metabolic disease.
1983; 6(3):133-4. doi:
10.1007/bf01800748. [PMID: 6422148] - R G Gray, S E Hill, R J Pollitt. Reduced ornithine catabolism in cultured fibroblasts and phytohaemagglutinin-stimulated lymphocytes from a patient with hyperornithinaemia, hyperammonaemia and homocitrullinuria.
Clinica chimica acta; international journal of clinical chemistry.
1982 Feb; 118(2-3):141-8. doi:
10.1016/0009-8981(82)90001-8. [PMID: 6120052] - V E Shih, R Mandell, A Herzfeld. Defective ornithine metabolism in cultured skin fibroblasts from patients with the syndrome of hyperornithinemia, hyperammonemia and homocitrullinuria.
Clinica chimica acta; international journal of clinical chemistry.
1982 Feb; 118(2-3):149-57. doi:
10.1016/0009-8981(82)90002-x. [PMID: 7055977] - R W Wilson, L E Dawson, A F Kohrman. A new metabolic product of L-homocitrulline.
Biochemical medicine.
1977 Aug; 18(1):80-6. doi:
10.1016/0006-2944(77)90052-7. [PMID: 901434] - P D Gatfield, E Taller, D M Wolfe, M D Haust. Hyperornithinemia, hyperammonemia, and homocitrullinuria associated with decreased carbamyl phosphate synthetase I activity.
Pediatric research.
1975 May; 9(5):488-97. doi:
10.1203/00006450-197505000-00006. [PMID: 166348] - W L RYAN, I C WELLS. HOMOCITRULLINE AND HOMOARGININE SYNTHESIS FROM LYSINE.
Science (New York, N.Y.).
1964 May; 144(3622):1122-7. doi:
10.1126/science.144.3622.1122. [PMID: 14148430] - T GERRITSEN, J G VAUGHN, H A WAISMAN. The origin of homocitrulline in the urine of infants.
Archives of biochemistry and biophysics.
1963 Feb; 100(?):298-301. doi:
10.1016/0003-9861(63)90076-6. [PMID: 13960562] - T GERRITSEN, H A WAISMAN, S H LIPTON, F M STRONG. Natural occurrence of homocitrulline. I. Excretion in the urine.
Archives of biochemistry and biophysics.
1962 Apr; 97(?):34-6. doi:
10.1016/0003-9861(62)90040-1. [PMID: 13898044] - T GERRITSEN, S H LIPTON, F M STRONG, H A WAISMAN. On the isolation and identification of homocitrulline from urine.
Biochemical and biophysical research communications.
1961 Apr; 4(?):379-83. doi:
10.1016/0006-291x(61)90222-4. [PMID: 13704730]
