74005
DL-3-Ureidoisobutyric acid
≥99.0% (HPLC)
别名:
β-UBA, β-Ureidoisobutyric acid, 3-(Carbamoylamino)-2-methylpropanoic acid, 3-Ureido-2-methylpropanoic acid, 3-[(Aminocarbonyl)amino]-2-methylpropanoic acid, N-Carbamoyl-2-methyl-β-alanine
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关于此项目
经验公式（希尔记法）:
C5H10N2O3
化学文摘社编号:
分子量:
146.14
UNSPSC Code:
41116107
NACRES:
NA.24
PubChem Substance ID:
MDL number:
Beilstein/REAXYS Number:
1768736
InChI key
PHENTZNALBMCQD-UHFFFAOYSA-N
SMILES string
OC(C(C)CNC(N)=O)=O
InChI
1S/C5H10N2O3/c1-3(4(8)9)2-7-5(6)10/h3H,2H2,1H3,(H,8,9)(H3,6,7,10)
assay
≥99.0% (HPLC)
technique(s)
HPLC: suitable, gas chromatography (GC): suitable
format
neat
storage temp.
2-8°C
Quality Level
Biochem/physiol Actions
3-Ureidoisobutyrate 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.
signalword
Danger
hcodes
Hazard Classifications
Eye Dam. 1
存储类别
11 - Combustible Solids
wgk
WGK 3
flash_point_f
Not applicable
flash_point_c
Not applicable
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.
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
André B P van Kuilenburg et al.
Human molecular genetics, 13(22), 2793-2801 (2004-09-24)
beta-Ureidopropionase deficiency is an inborn error of the pyrimidine degradation pathway, affecting the cleavage of N-carbamyl-beta-alanine and N-carbamyl-beta-aminoisobutyric acid. In this study, we report the elucidation of the genetic basis underlying a beta-ureidopropionase deficiency in four patients presenting with neurological
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