方案
≥85% (HPLC)
质量水平
旋光性
[α]/D -19±2°, c = 0.1 in ethanol
包装形式
neat
储存温度
2-8°C
SMILES字符串
O=C(O[H])[C@@](C([H])([H])[H])([H])C([H])([H])N([H])C(N([H])[H])=O
生化/生理作用
This metabolite is increased in the urine of patients with beta-ureidopropionase deficiency and can be used to predict patient′s individual phenotypes of enzyme deficiencies in pyrimidine metabolism when associated with a risk for severe toxicity against the antineoplastic agent 5-fluorouracil.
储存分类代码
11 - Combustible Solids
WGK
WGK 3
闪点(°F)
Not applicable
闪点(°C)
Not applicable
NMR-based urinalysis for rapid diagnosis of ?-ureido- propionase deficiency in a patient with Dravet syndrome.
Lam, C.W., et al.
Clinica Chimica Acta; International Journal of Clinical Chemistry, 440, 201-204 (2015)
Urinary metabonomics of stomach cancer assessed by rapid resolution liquid chromatography/time-of-flight mass spectrometry
Zhang, Y., et al.
Chinese Medical Journal (English Edition), 126, 1930-1933 (2013)
Ute Hofmann et al.
Journal of chromatography. B, Analytical technologies in the biomedical and life sciences, 791(1-2), 371-380 (2003-06-12)
Enzyme deficiencies in pyrimidine metabolism are associated with a risk for severe toxicity against the antineoplastic agent 5-fluorouracil. To assess whether urinary levels of pyrimidines and their metabolites can be used for predicting patients' individual phenotype, a new gas chromatographic-tandem
Morimasa Ohse et al.
Journal of mass spectrometry : JMS, 37(9), 954-962 (2002-09-25)
Dihydropyrimidine dehydrogenase (DHPDase), dihydropyrimidinase (DHPase) and beta-ureidopropionase (betaUPase) are the enzymes that catalyze the first, second, and third steps of the degradation of pyrimidines, respectively. beta-Ureidopropionate (betaUP) and beta-ureidoisobutyrate (betaUIB) are increased in the urine of patients with betaUPase deficiency.
André B P van Kuilenburg et al.
Biochimica et biophysica acta, 1822(7), 1096-1108 (2012-04-25)
ß-ureidopropionase is the third enzyme of the pyrimidine degradation pathway and catalyzes the conversion of N-carbamyl-ß-alanine and N-carbamyl-ß-aminoisobutyric acid to ß-alanine and ß-aminoisobutyric acid, ammonia and CO(2). To date, only five genetically confirmed patients with a complete ß-ureidopropionase deficiency have
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