Research Center for Genetic Medicine, Children's National Medical Center, The George Washington University, Washington, DC 20010, USA.
Mol Genet Metab. 2009 Dec;98(4):325-30. doi: 10.1016/j.ymgme.2009.07.010. Epub 2009 Jul 14.
We studied the effect on ureagenesis of a single dose of N-carbamylglutamate (NCG) in healthy young adults who received a constant infusion (300 min) of NaH(13)CO(3). Isotope ratio-mass spectrometry was used to measure the appearance of label in [(13)C]urea. At 90 min after initiating the H(13)CO3-infusion each subject took a single dose of NCG (50 mg/kg). In 5/6 studies the administration of NCG increased the formation of [(13)C]urea. Treatment with NCG significantly diminished the concentration of blood alanine, but not that of glutamine or arginine. The blood glucose concentration was unaffected by NCG administration. No untoward side effects were observed. The data indicate that treatment with NCG stimulates ureagenesis and could be useful in clinical settings of acute hyperammonemia of various etiologies.
我们研究了在接受 NaH(13)CO(3)恒速输注(300 分钟)的健康年轻成年人中单次给予 N-氨甲酰谷氨酸(NCG)对尿素生成的影响。使用同位素比质谱法测量 [(13)C]尿素中标记的出现。在开始 H(13)CO3 输注后 90 分钟,每位受试者单次给予 NCG(50 mg/kg)。在 6 项研究中的 5 项中,NCG 的给药增加了 [(13)C]尿素的形成。NCG 治疗显著降低了血液丙氨酸的浓度,但不降低谷氨酰胺或精氨酸的浓度。血糖浓度不受 NCG 给药的影响。未观察到不良反应。数据表明,NCG 治疗可刺激尿素生成,可在各种病因的急性高氨血症的临床环境中使用。