3-酰脲丙酸

Anal. Biochem. 122(2) , 345-7, (1982)

J. Chromatogr. B. Analyt. Technol. Biomed. Life Sci. 791(1-2) , 371-80, (2003)

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 ...

J. Inherit. Metab. Dis. 28(6) , 1109-22, (2005)

To evaluate the significance of inborn metabolic disorders of the pyrimidine degradation pathway, 450 children with unspecific neurological symptoms were comprehensively studied; 200 healthy children were recruited as controls. Uracil and thymine as well as t...

J. Neurosci. Res. 66(4) , 666-73, (2001)

3-Ureidopropionate (3-UPA) is a physiologic metabolite in pyrimidine degradation. Pathological accumulation of 3-UPA in body fluids is found in 3-ureidopropionase deficiency and severe forms of propionic aciduria. Both diseases clinically present with a sever...

Anal. Biochem. 188(1) , 233-6, (1990)

A sensitive assay for beta-ureidopropionase based on derivatization of the reaction product beta-alanine with phenylisothiocyanate has been developed. Purification of the resulting phenylthiocarbamoyl-beta-alanine is achieved on a LiChrospher 100 C18 reversed...

Adv. Exp. Med. Biol. 309B , 15-9, (1991)

Magn. Reson. Med. 46(5) , 1014-7, (2001)

In this work, NMR investigations that led to the discovery of a new inborn error of metabolism, beta-ureidopropionase (UP) deficiency, are reported. 1D (1)H-NMR experiments were performed using a patient's urine. 3-Ureidopropionic acid was observed in elevate...

J. Chromatogr. B. Analyt. Technol. Biomed. Life Sci. 839(1-2) , 45-53, (2006)

A liquid chromatography-tandem mass spectrometric assay for the determination of uracil, 5,6-dihydrouracil and beta-ureidopropionic acid in urine was developed to measure the activities of enzymes involved in pyrimidine breakdown. The assay was required to in...

Cancer Res. 45(11 Pt 1) , 5405-12, (1985)

Enzymes of the pyrimidine base catabolism, dihydrouracil dehydrogenase (EC 1.3.1.2), dihydropyrimidinase (EC 3.5.2.2), and beta-ureidopropionase (EC 3.5.1.6) were compared in the cytosolic extract of several normal and neoplastic human tissues. The activity w...

Mol. Genet. Metab. 91(2) , 157-64, (2007)

Dihydropyrimidinase (DHP) is the second enzyme of the pyrimidine degradation pathway and it catalyses the ring opening of 5,6-dihydrouracil and 5,6-dihydrothymine to N-carbamyl-beta-alanine and N-carbamyl-beta-aminoisobutyric acid, respectively. To date, only...