推荐产品
product name
N-氨基甲酰-L-谷氨酸,
化驗
≥98.0% (TLC)
形狀
powder
顏色
white
應用
cell analysis
儲存溫度
2-8°C
SMILES 字串
NC(=O)N[C@@H](CCC(O)=O)C(O)=O
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
InChI 密鑰
LCQLHJZYVOQKHU-VKHMYHEASA-N
基因資訊
human ... CPS1(1373)
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生化/生理作用
N-氨基甲酰-L-谷氨酸(氨甲酰谷氨酸)被用于研究其作为替代疗法对N-乙酰谷氨酸合成酶(NAGS)缺乏症的治疗潜力,并用于治疗高氨血症。
儲存類別代碼
11 - Combustible Solids
水污染物質分類(WGK)
WGK 3
閃點(°F)
Not applicable
閃點(°C)
Not applicable
個人防護裝備
dust mask type N95 (US), Eyeshields, Gloves
Neonatology, 97(3), 286-290 (2009-11-06)
In propionic aciduria and methylmalonic aciduria, hyperammonemia as a symptom of metabolic decompensation is one of the major clinical problems. Hyperammonemia is a true neonatal emergency with high mortality and neurological complications in most survivors. It requires a rapid and
Amino acids, 39(3), 831-839 (2010-03-10)
This study determined effects of dietary supplementation with L-arginine (Arg) or N-carbamylglutamate (NCG) on intestinal health and growth in early-weaned pigs. Eighty-four Landrace x Yorkshire pigs (average body weight of 5.56+/-0.07 kg; weaned at 21 days of age) were fed
Pediatrics, 126(1), e208-e214 (2010-06-23)
The objective of this study was to determine whether N-carbamylglutamate (NCG) reduces plasma levels of ammonia and glutamine and increases the rate of ureagenesis in patients with propionic acidemia (PA). Identical 4-hour studies were performed before and immediately after a
Metabolic brain disease, 24(3), 409-414 (2009-08-19)
Maple syrup urine disease (MSUD) is a defect in the catabolism of the branched-chain amino acids; leucine, isoleucine, and valine. Affected patients may also develop hyperammonaemia of unknown etiology. This report describes a four-year-old girl with MSUD, who presented with
The Journal of pediatrics, 147(2), 260-262 (2005-08-30)
In a prospective study, patients with a suspected urea cycle defect underwent oral N-carbamoyl-L-glutamic acid loading testing. In patients with subsequently confirmed N-acetylglutamate synthase deficiency, hyperammonemia normalized within 8 hours. This test may be useful in the early diagnosis of
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