Carglumic Acid (BioDeep_00001867894)
Main id: BioDeep_00000003189
Volatile Flavor Compounds
代谢物信息卡片
Carglumic Acid
化学式: C6H10N2O5 (190.058969)
中文名称: N-氨基甲酰基- L -谷氨酸
谱图信息:
最多检出来源 () 0%
分子结构信息
SMILES: C(CC(=O)O)C(C(=O)O)NC(=O)N
InChI: InChI=1S/C6H10N2O5/c7-6(13)8-3(5(11)12)1-2-4(9)10/h3H,1-2H2,(H,9,10)(H,11,12)(H3,7,8,13)/t3-/m0/s1
描述信息
A - Alimentary tract and metabolism > A16 - Other alimentary tract and metabolism products > A16A - Other alimentary tract and metabolism products > A16AA - Amino acids and derivatives
C78275 - Agent Affecting Blood or Body Fluid
同义名列表
2 个代谢物同义名
数据库引用编号
13 个数据库交叉引用编号
- ChEBI: CHEBI:71028
- KEGG: C05829
- KEGGdrug: D07130
- PubChem: 121396
- DrugBank: DB06775
- ChEMBL: CHEMBL1201780
- CAS: 1188-38-1
- ChEBI: CHEBI:190595
- PubChem: 8123
- 3DMET: B00858
- NIKKAJI: J2.088.972A
- RefMet: Carglumic acid
- KNApSAcK: 71028
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)
0 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
- Qiulin Liu, Jiabo Li, Di Han, Jinfeng Wang, Jian Zheng, Wei Ma, Chunqiang Wang. N-carbamylglutamate supplementation induces functional egg production in layers by modulating liver transcriptome profiles.
Poultry science.
2024 Jan; 103(1):103223. doi:
10.1016/j.psj.2023.103223. [PMID: 38035861] - F F Gu, L Y Jiang, D M Wang, F Q Zhao, J X Liu. Supplementation with N-carbamoylglutamate during the transition period improves the function of neutrophils and reduces inflammation and oxidative stress in dairy cows.
Journal of dairy science.
2022 Jul; 105(7):5786-5795. doi:
10.3168/jds.2021-21159. [PMID: 35637005] - P Sonaimuthu, E Senkevitch, N Haskins, P Uapinyoying, M McNutt, H Morizono, M Tuchman, L Caldovic. Gene delivery corrects N-acetylglutamate synthase deficiency and enables insights in the physiological impact of L-arginine activation of N-acetylglutamate synthase.
Scientific reports.
2021 02; 11(1):3580. doi:
10.1038/s41598-021-82994-8. [PMID: 33574402] - Shangli Wei, Yuze Yang, Yinxia Xiao, Yan Liu, Jianhui Tian, Leon J Spicer, Tao Feng. Effects of N-carbamylglutamate on steroidogenesis and relative abundances of mRNA transcripts in pig placental trophoblasts.
Animal reproduction science.
2020 Oct; 221(?):106569. doi:
10.1016/j.anireprosci.2020.106569. [PMID: 32861119] - Yanfen Ma, Jinwei Yao, Shuo Zhou, Yuling Mi, Jian Li, Caiqiao Zhang. Improvement of eggshell quality by dietary N-carbamylglutamate supplementation in laying chickens.
Poultry science.
2020 Aug; 99(8):4085-4095. doi:
10.1016/j.psj.2020.04.004. [PMID: 32731996] - Katrine D Galsgaard, Jens Pedersen, Sasha A S Kjeldsen, Marie Winther-Sørensen, Elena Stojanovska, Hendrik Vilstrup, Cathrine Ørskov, Nicolai J Wewer Albrechtsen, Jens J Holst. Glucagon receptor signaling is not required for N-carbamoyl glutamate- and l-citrulline-induced ureagenesis in mice.
American journal of physiology. Gastrointestinal and liver physiology.
2020 05; 318(5):G912-G927. doi:
10.1152/ajpgi.00294.2019. [PMID: 32174131] - Yuming Wang, Shuaijuan Han, Junyan Zhou, Peili Li, Gang Wang, Haitao Yu, Shuang Cai, Xiangfang Zeng, Lee J Johnston, Crystal L Levesque, Shiyan Qiao. Effects of dietary crude protein level and N-carbamylglutamate supplementation on nutrient digestibility and digestive enzyme activity of jejunum in growing pigs.
Journal of animal science.
2020 Apr; 98(4):. doi:
10.1093/jas/skaa088. [PMID: 32201878] - Yanfen Ma, Shuo Zhou, Xin Lin, Weidong Zeng, Yuling Mi, Caiqiao Zhang. Effect of dietary N-carbamylglutamate on development of ovarian follicles via enhanced angiogenesis in the chicken.
Poultry science.
2020 Jan; 99(1):578-589. doi:
NULL. [PMID: 32416844] - Hao Zhang, Hua Sun, Along Peng, Shuang Guo, Mengzhi Wang, Juan J Loor, Hongrong Wang. N-carbamylglutamate and l-arginine promote intestinal function in suckling lambs with intrauterine growth restriction by regulating antioxidant capacity via a nitric oxide-dependent pathway.
Food & function.
2019 Oct; 10(10):6374-6384. doi:
10.1039/c9fo01752f. [PMID: 31508643] - T Feng, A A DeVore, M C Perego, B C Morrell, L J Spicer. Effects of N-carbamylglutamate and arginine on steroidogenesis and proliferation of pig granulosa cells in vitro.
Animal reproduction science.
2019 Oct; 209(?):106138. doi:
10.1016/j.anireprosci.2019.106138. [PMID: 31514935] - H Y Huang, P Chen, X F Liang, X F Wu, C P Wang, X Gu, M Xue. Dietary N-Carbamylglutamate (NCG) alleviates liver metabolic disease and hepatocyte apoptosis by suppressing ERK1/2-mTOR-S6K1 signal pathway via promoting endogenous arginine synthesis in Japanese seabass (Lateolabrax japonicus).
Fish & shellfish immunology.
2019 Jul; 90(?):338-348. doi:
10.1016/j.fsi.2019.04.294. [PMID: 31075404] - Hao Zhang, Along Peng, Yin Yu, Shuang Guo, Mengzhi Wang, Danielle N Coleman, Juan J Loor, Hongrong Wang. N-Carbamylglutamate and l-Arginine Promote Intestinal Absorption of Amino Acids by Regulating the mTOR Signaling Pathway and Amino Acid and Peptide Transporters in Suckling Lambs with Intrauterine Growth Restriction.
The Journal of nutrition.
2019 06; 149(6):923-932. doi:
10.1093/jn/nxz016. [PMID: 31149712] - Yan Hu, Dan Shao, Qiang Wang, Yunqi Xiao, Xu Zhao, Yiru Shen, Shan Zhang, Haibing Tong, Shourong Shi. Effects of dietary N-carbamylglutamate supplementation on growth performance, tissue development and blood parameters of yellow-feather broilers.
Poultry science.
2019 May; 98(5):2241-2249. doi:
10.3382/ps/pey591. [PMID: 30668818] - Hao Zhang, Along Peng, Shuang Guo, Mengzhi Wang, Juan J Loor, Hongrong Wang. Dietary N-carbamylglutamate and l-arginine supplementation improves intestinal energy status in intrauterine-growth-retarded suckling lambs.
Food & function.
2019 Apr; 10(4):1903-1914. doi:
10.1039/c8fo01618f. [PMID: 30869672] - Liansheng Wang, Jinnan Li, Chang'an Wang, Zhigang Zhao, Liang Luo, Xue Du, Qiyou Xu. Effect of N-carbamoylglutamate supplementation on the growth performance, antioxidant status and immune response of mirror carp (Cyprinus carpio) fed an arginine-deficient diet.
Fish & shellfish immunology.
2019 Jan; 84(?):280-289. doi:
10.1016/j.fsi.2018.10.001. [PMID: 30291983] - F F Gu, S L Liang, Z H Wei, C P Wang, H Y Liu, J X Liu, D M Wang. Short communication: Effects of dietary addition of N-carbamoylglutamate on milk composition in mid-lactating dairy cows.
Journal of dairy science.
2018 Dec; 101(12):10985-10990. doi:
10.3168/jds.2018-15162. [PMID: 30268601] - Lingwei Sun, Hao Zhang, Ziyu Wang, Yixuan Fan, Yixuan Guo, Feng Wang. Dietary rumen-protected arginine and N-carbamylglutamate supplementation enhances fetal growth in underfed ewes.
Reproduction, fertility, and development.
2018 Jul; 30(8):1116-1127. doi:
10.1071/rd17164. [PMID: 31039923] - Shuang Cai, Jinlong Zhu, Xiangzhou Zeng, Qianhong Ye, Changchuan Ye, Xiangbing Mao, Shihai Zhang, Shiyan Qiao, Xiangfang Zeng. Maternal N-Carbamylglutamate Supply during Early Pregnancy Enhanced Pregnancy Outcomes in Sows through Modulations of Targeted Genes and Metabolism Pathways.
Journal of agricultural and food chemistry.
2018 Jun; 66(23):5845-5852. doi:
10.1021/acs.jafc.8b01637. [PMID: 29804448] - Albina Tummolo, Livio Melpignano, Antonella Carella, Anna Maria Di Mauro, Elvira Piccinno, Marcella Vendemiale, Federica Ortolani, Stefania Fedele, Maristella Masciopinto, Francesco Papadia. Long-term continuous N-carbamylglutamate treatment in frequently decompensated propionic acidemia: a case report.
Journal of medical case reports.
2018 Apr; 12(1):103. doi:
10.1186/s13256-018-1631-1. [PMID: 29679984] - T Feng, L F Schütz, B C Morrell, M C Perego, L J Spicer. Effects of N-carbamylglutamate and L-arginine on steroidogenesis and gene expression in bovine granulosa cells.
Animal reproduction science.
2018 Jan; 188(?):85-92. doi:
10.1016/j.anireprosci.2017.11.012. [PMID: 29150243] - Jorge Y P Palencia, Alysson Saraiva, Márvio Lobão Teixeira Abreu, Marcio G Zangeronimo, Allan P Schinckel, Cesar Augusto Pospissil Garbossa. Effectiveness of citrulline and N-carbamoyl glutamate as arginine precursors on reproductive performance in mammals: A systematic review.
PloS one.
2018; 13(12):e0209569. doi:
10.1371/journal.pone.0209569. [PMID: 30571792] - Cristina Menni, Jonas Zierer, Tess Pallister, Matthew A Jackson, Tao Long, Robert P Mohney, Claire J Steves, Tim D Spector, Ana M Valdes. Omega-3 fatty acids correlate with gut microbiome diversity and production of N-carbamylglutamate in middle aged and elderly women.
Scientific reports.
2017 09; 7(1):11079. doi:
10.1038/s41598-017-10382-2. [PMID: 28894110] - L Sun, H Zhang, Y Fan, Y Guo, G Zhang, H Nie, F Wang. Metabolomic profiling in umbilical venous plasma reveals effects of dietary rumen-protected arginine or N-carbamylglutamate supplementation in nutrient-restricted Hu sheep during pregnancy.
Reproduction in domestic animals = Zuchthygiene.
2017 Jun; 52(3):376-388. doi:
10.1111/rda.12919. [PMID: 28220550] - Anne H O'Donnell-Luria, Alexander P Lin, Sai K Merugumala, Frances Rohr, Susan E Waisbren, Rebecca Lynch, Vatche Tchekmedyian, Aaron D Goldberg, Andrew Bellinger, J Ricardo McFaline-Figueroa, Tracey Simon, Esteban F Gershanik, Bruce D Levy, David E Cohen, Martin A Samuels, Gerard T Berry, Natasha Y Frank. Brain MRS glutamine as a biomarker to guide therapy of hyperammonemic coma.
Molecular genetics and metabolism.
2017 05; 121(1):9-15. doi:
10.1016/j.ymgme.2017.03.003. [PMID: 28408159] - Birgitta C Burckhardt, Gerhard Burckhardt. Interaction of Excitatory Amino Acid Transporters 1 - 3 (EAAT1, EAAT2, EAAT3) with N-Carbamoylglutamate and N-Acetylglutamate.
Cellular physiology and biochemistry : international journal of experimental cellular physiology, biochemistry, and pharmacology.
2017; 43(5):1907-1916. doi:
10.1159/000484110. [PMID: 29055942] - Guangmang Liu, Wei Cao, Tingting Fang, Gang Jia, Hua Zhao, Xiaoling Chen, Caimei Wu, Jing Wang. Urinary Metabolomic Approach Provides New Insights into Distinct Metabolic Profiles of Glutamine and N-Carbamylglutamate Supplementation in Rats.
Nutrients.
2016 Aug; 8(8):. doi:
10.3390/nu8080478. [PMID: 27527211] - Hao Zhang, Lingwei Sun, Ziyu Wang, Mingtian Deng, Haitao Nie, Guomin Zhang, Tiewei Ma, Feng Wang. N-carbamylglutamate and L-arginine improved maternal and placental development in underfed ewes.
Reproduction (Cambridge, England).
2016 06; 151(6):623-35. doi:
10.1530/rep-16-0067. [PMID: 26980807] - Wei Cao, Liang Xiao, Guangmang Liu, Tingting Fang, Xianjian Wu, Gang Jia, Hua Zhao, Xiaoling Chen, Caimei Wu, Jingyi Cai, Jing Wang. Dietary arginine and N-carbamylglutamate supplementation enhances the antioxidant statuses of the liver and plasma against oxidative stress in rats.
Food & function.
2016 May; 7(5):2303-11. doi:
10.1039/c5fo01194a. [PMID: 27109002] - H Zhang, L W Sun, Z Y Wang, M T Deng, G M Zhang, R H Guo, T W Ma, F Wang. Dietary -carbamylglutamate and rumen-protected -arginine supplementation ameliorate fetal growth restriction in undernourished ewes.
Journal of animal science.
2016 May; 94(5):2072-85. doi:
10.2527/jas.2015-9587. [PMID: 27285704] - Guangmang Liu, Liang Xiao, Wei Cao, Tingting Fang, Gang Jia, Xiaoling Chen, Hua Zhao, Caimei Wu, Jing Wang. Changes in the metabolome of rats after exposure to arginine and N-carbamylglutamate in combination with diquat, a compound that causes oxidative stress, assessed by 1H NMR spectroscopy.
Food & function.
2016 Feb; 7(2):964-74. doi:
10.1039/c5fo01486g. [PMID: 26732548] - Sufin Yap, Huey Yin Leong, Fadzlina Abdul Aziz, Haszlin Hassim, Pavai Sthaneshwar, Ser Huy Teh, Ili Syazwana Abdullah, Lock Hock Ngu, Zulqarnain Mohamed. N-Carbamylglutamate Is an Effective Treatment for Acute Neonatal Hyperammonaemia in a Patient with Methylmalonic Aciduria.
Neonatology.
2016; 109(4):303-7. doi:
10.1159/000443630. [PMID: 26907495] - Jinlong Zhu, Xiangfang Zeng, Qian Peng, Shenming Zeng, Haiyi Zhao, Hexiao Shen, Shiyan Qiao. Maternal N-Carbamylglutamate Supplementation during Early Pregnancy Enhances Embryonic Survival and Development through Modulation of the Endometrial Proteome in Gilts.
The Journal of nutrition.
2015 Oct; 145(10):2212-20. doi:
10.3945/jn.115.216333. [PMID: 26290006] - Elisabeth Schwob, Yohannes Hagos, Gerhard Burckhardt, Birgitta C Burckhardt. Transporters involved in renal excretion of N-carbamoylglutamate, an orphan drug to treat inborn n-acetylglutamate synthase deficiency.
American journal of physiology. Renal physiology.
2014 Dec; 307(12):F1373-9. doi:
10.1152/ajprenal.00482.2014. [PMID: 25354943] - B Zhang, L Q Che, Y Lin, Y Zhuo, Z F Fang, S Y Xu, J Song, Y S Wang, Y Liu, P Wang, D Wu. Effect of dietary N-carbamylglutamate levels on reproductive performance of gilts.
Reproduction in domestic animals = Zuchthygiene.
2014 Oct; 49(5):740-5. doi:
10.1111/rda.12358. [PMID: 25039302] - Nicholas Ah Mew, Robert McCarter, Yevgeny Daikhin, Uta Lichter-Konecki, Ilana Nissim, Marc Yudkoff, Mendel Tuchman. Augmenting ureagenesis in patients with partial carbamyl phosphate synthetase 1 deficiency with N-carbamyl-L-glutamate.
The Journal of pediatrics.
2014 Aug; 165(2):401-403.e3. doi:
10.1016/j.jpeds.2014.04.012. [PMID: 24880889] - B Chacher, W Zhu, J A Ye, D M Wang, J X Liu. Effect of dietary N-carbamoylglutamate on milk production and nitrogen utilization in high-yielding dairy cows.
Journal of dairy science.
2014; 97(4):2338-45. doi:
10.3168/jds.2013-7330. [PMID: 24485674] - H S Yang, D Z Fu, X F Kong, W C Wang, X J Yang, C M Nyachoti, Y L Yin. Dietary supplementation with N-carbamylglutamate increases the expression of intestinal amino acid transporters in weaned Huanjiang mini-pig piglets.
Journal of animal science.
2013 Jun; 91(6):2740-8. doi:
10.2527/jas.2012-5795. [PMID: 23478823] - Albina Tummolo, Vito Favia, Rosa Bellantuono, Vito Bellino, Antonio Ranieri, Amelia Morrone, Tommaso De Palo, Francesco Papadia. Successful early management of a female patient with a metabolic stroke due to ornithine transcarbamylase deficiency.
Pediatric emergency care.
2013 May; 29(5):656-8. doi:
10.1097/pec.0b013e31828ec2b9. [PMID: 23640148] - X D Liu, X Wu, Y L Yin, Y Q Liu, M M Geng, H S Yang, Francois Blachier, G Y Wu. Effects of dietary L-arginine or N-carbamylglutamate supplementation during late gestation of sows on the miR-15b/16, miR-221/222, VEGFA and eNOS expression in umbilical vein.
Amino acids.
2012 Jun; 42(6):2111-9. doi:
10.1007/s00726-011-0948-5. [PMID: 21638020] - X Wu, Y L Yin, Y Q Liu, X D Liu, Z Q Liu, T J Li, R L Huang, Z Ruan, Z Y Deng. Effect of dietary arginine and N-carbamoylglutamate supplementation on reproduction and gene expression of eNOS, VEGFA and PlGF1 in placenta in late pregnancy of sows.
Animal reproduction science.
2012 Jun; 132(3-4):187-92. doi:
10.1016/j.anireprosci.2012.05.002. [PMID: 22682770] - Emilee Senkevitch, Juan Cabrera-Luque, Hiroki Morizono, Ljubica Caldovic, Mendel Tuchman. A novel biochemically salvageable animal model of hyperammonemia devoid of N-acetylglutamate synthase.
Molecular genetics and metabolism.
2012 Jun; 106(2):160-8. doi:
10.1016/j.ymgme.2012.03.004. [PMID: 22503289] - Xiangfang Zeng, Zhimin Huang, Xiangbing Mao, Junjun Wang, Guoyao Wu, Shiyan Qiao. N-carbamylglutamate enhances pregnancy outcome in rats through activation of the PI3K/PKB/mTOR signaling pathway.
PloS one.
2012; 7(7):e41192. doi:
10.1371/journal.pone.0041192. [PMID: 22848442] - Cigdem Seher Kasapkara, Fatih Suheyl Ezgu, Ilyas Okur, Leyla Tumer, Gursel Biberoglu, Alev Hasanoglu. N-carbamylglutamate treatment for acute neonatal hyperammonemia in isovaleric acidemia.
European journal of pediatrics.
2011 Jun; 170(6):799-801. doi:
10.1007/s00431-010-1362-9. [PMID: 21207059] - Marta Daniotti, Giancarlo la Marca, Patrizio Fiorini, Luca Filippi. New developments in the treatment of hyperammonemia: emerging use of carglumic acid.
International journal of general medicine.
2011 Jan; 4(?):21-8. doi:
10.2147/ijgm.s10490. [PMID: 21403788] - Johannes Häberle. Role of carglumic acid in the treatment of acute hyperammonemia due to N-acetylglutamate synthase deficiency.
Therapeutics and clinical risk management.
2011; 7(?):327-32. doi:
10.2147/tcrm.s12703. [PMID: 21941437] - Xin Wu, Zheng Ruan, Yunling Gao, Yulong Yin, Xihong Zhou, Lei Wang, Meimei Geng, Yongqing Hou, Guoyao Wu. Dietary supplementation with L-arginine or N-carbamylglutamate enhances intestinal growth and heat shock protein-70 expression in weanling pigs fed a corn- and soybean meal-based diet.
Amino acids.
2010 Aug; 39(3):831-9. doi:
10.1007/s00726-010-0538-y. [PMID: 20213438] - Nicholas Ah Mew, Robert McCarter, Yevgeny Daikhin, Itzhak Nissim, Marc Yudkoff, Mendel Tuchman. N-carbamylglutamate augments ureagenesis and reduces ammonia and glutamine in propionic acidemia.
Pediatrics.
2010 Jul; 126(1):e208-14. doi:
10.1542/peds.2010-0008. [PMID: 20566609] - Luca Filippi, Elena Gozzini, Patrizio Fiorini, Sabrina Malvagia, Giancarlo la Marca, Maria Alice Donati. N-carbamylglutamate in emergency management of hyperammonemia in neonatal acute onset propionic and methylmalonic aciduria.
Neonatology.
2010; 97(3):286-90. doi:
10.1159/000255168. [PMID: 19887858] - Sema Kalkan Ucar, Mahmut Coker, Sara Habif, Eylem Ulas Saz, Bulent Karapinar, Hakan Ucar, Omer Kitis, Marinus Duran. The first use of N-carbamylglutamate in a patient with decompensated maple syrup urine disease.
Metabolic brain disease.
2009 Sep; 24(3):409-14. doi:
10.1007/s11011-009-9155-4. [PMID: 19688253] - S Jones, C A B Reed, S Vijay, J H Walter, A A M Morris. N-carbamylglutamate for neonatal hyperammonaemia in propionic acidaemia.
Journal of inherited metabolic disease.
2008 Dec; 31 Suppl 2(?):S219-22. doi:
10.1007/s10545-008-0777-1. [PMID: 18338235] - Mendel Tuchman, Ljubica Caldovic, Yevgeny Daikhin, Oksana Horyn, Ilana Nissim, Itzhak Nissim, Mark Korson, Barbara Burton, Marc Yudkoff. N-carbamylglutamate markedly enhances ureagenesis in N-acetylglutamate deficiency and propionic acidemia as measured by isotopic incorporation and blood biomarkers.
Pediatric research.
2008 Aug; 64(2):213-7. doi:
10.1203/pdr.0b013e318179454b. [PMID: 18414145] - Rolf Lindemann, Mia C Myhre, Mari Bakken, Drude Fugelseth, Cecilie F Rustad, Berit Woldseth. [A newborn infant with hyperventilation].
Tidsskrift for den Norske laegeforening : tidsskrift for praktisk medicin, ny raekke.
2008 Jun; 128(13):1535-6. doi:
NULL. [PMID: 18604903] - Virginie Levrat, Isabelle Forest, Alain Fouilhoux, Cécile Acquaviva, Christine Vianey-Saban, Nathalie Guffon. Carglumic acid: an additional therapy in the treatment of organic acidurias with hyperammonemia?.
Orphanet journal of rare diseases.
2008 Jan; 3(?):2. doi:
10.1186/1750-1172-3-2. [PMID: 18234091] - A Nordenström, M Halldin, B Hallberg, J Alm. A trial with N-carbamylglutamate may not detect all patients with NAGS deficiency and neonatal onset.
Journal of inherited metabolic disease.
2007 Jun; 30(3):400. doi:
10.1007/s10545-007-0454-9. [PMID: 17510757] - Jason W Frank, Jeffery Escobar, Hanh V Nguyen, Scott C Jobgen, Wenjuan S Jobgen, Teresa A Davis, Guoyao Wu. Oral N-carbamylglutamate supplementation increases protein synthesis in skeletal muscle of piglets.
The Journal of nutrition.
2007 Feb; 137(2):315-9. doi:
10.1093/jn/137.2.315. [PMID: 17237304] - B Gebhardt, S Dittrich, S Parbel, S Vlaho, O Matsika, H Bohles. N-carbamylglutamate protects patients with decompensated propionic aciduria from hyperammonaemia.
Journal of inherited metabolic disease.
2005; 28(2):241-4. doi:
10.1007/s10545-005-5260-7. [PMID: 15877213] - Ljubica Caldovic, Hiroki Morizono, Yevgeny Daikhin, Itzhak Nissim, Robert J McCarter, Marc Yudkoff, Mendel Tuchman. Restoration of ureagenesis in N-acetylglutamate synthase deficiency by N-carbamylglutamate.
The Journal of pediatrics.
2004 Oct; 145(4):552-4. doi:
10.1016/j.jpeds.2004.06.047. [PMID: 15480384] - Boris Gebhardt, Stefan Vlaho, Doris Fischer, Adrian Sewell, Hansjosef Böhles. N-carbamylglutamate enhances ammonia detoxification in a patient with decompensated methylmalonic aciduria.
Molecular genetics and metabolism.
2003 Aug; 79(4):303-4. doi:
10.1016/s1096-7192(03)00095-7. [PMID: 12948747] - P De Lonlay, C Benelli, F Fouque, A Ganguly, B Aral, C Dionisi-Vici, G Touati, C Heinrichs, D Rabier, P Kamoun, J J Robert, C Stanley, J M Saudubray. Hyperinsulinism and hyperammonemia syndrome: report of twelve unrelated patients.
Pediatric research.
2001 Sep; 50(3):353-7. doi:
10.1203/00006450-200109000-00010. [PMID: 11518822] - E Zammarchi, L Filippi, E Novembre, M A Donati. Biochemical evaluation of a patient with a familial form of leucine-sensitive hypoglycemia and concomitant hyperammonemia.
Metabolism: clinical and experimental.
1996 Aug; 45(8):957-60. doi:
10.1016/s0026-0495(96)90262-0. [PMID: 8769351] - N Guffon, C Vianey-Saban, J Bourgeois, D Rabier, J P Colombo, P Guibaud. A new neonatal case of N-acetylglutamate synthase deficiency treated by carbamylglutamate.
Journal of inherited metabolic disease.
1995; 18(1):61-5. doi:
10.1007/bf00711374. [PMID: 7623444] - E Grau, V Felipo, M D Miñana, S Grisolía. Treatment of hyperammonemia with carbamylglutamate in rats.
Hepatology (Baltimore, Md.).
1992 Mar; 15(3):446-8. doi:
10.1002/hep.1840150315. [PMID: 1544625]