Beta-ureidopropionase
Definition:
References:
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[1]. T Sakamoto, et al. Expression and properties of human liver beta-ureidopropionase. J Nutr Sci Vitaminol (Tokyo). 2001 Apr;47(2):132-8. [Content Brief]
[2]. P Vreken, et al. cDNA cloning, genomic structure and chromosomal localization of the human BUP-1 gene encoding beta-ureidopropionase. Biochim Biophys Acta. 1999 Oct 28;1447(2-3):251-7. [Content Brief]
[3]. André B P van Kuilenburg, et al. ß-ureidopropionase deficiency: phenotype, genotype and protein structural consequences in 16 patients. Biochim Biophys Acta. 2012 Jul;1822(7):1096-108. [Content Brief]
[4]. Dirk Maurer, et al. Crystal structure and pH-dependent allosteric regulation of human β-ureidopropionase, an enzyme involved in anticancer drug metabolism. Biochem J. 2018 Jul 31;475(14):2395-2416. [Content Brief]
[5]. A B Van Kuilenburg, et al. A radiochemical assay for beta-ureidopropionase using radiolabeled N-carbamyl-beta-alanine obtained via hydrolysis of [2-(14)C]5, 6-dihydrouracil. Anal Biochem. 1999 Aug 1;272(2):250-3. [Content Brief]
[6]. Yoko Nakajima, et al. Clinical, biochemical and molecular analysis of 13 Japanese patients with β-ureidopropionase deficiency demonstrates high prevalence of the c.977G > A (p.R326Q) mutation [corrected]. J Inherit Metab Dis. 2014 Sep;37(5):801-12. [Content Brief]
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