产品展示
DHP/Dihydropyrimidinase二氢嘧啶抗体
点击次数:249发布时间:2012/12/5 13:57:01
更新日期:2024/9/5 14:43:01
所 在 地:中国大陆
产品型号:BY-8287R
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货号:BY-8287R
英文名称:Anti-DHP/Dihydropyrimidinase
中文名称:二氢嘧啶抗体
其他名称:名DHP;DHPase;Dihydropyrimidinase;Dihydropyrimidine amidohydrolase;Dpys;DPYS_HUMAN;Hydantoinase.
抗体来源:Rabbit
克隆类型:polyclonal
蛋白分子量:
纯化方法:affinity purified by Protein A
交叉反应:Human
产品介绍:Catalyzes the second step of the reductive pyrimidine degradation, the reversible hydrolytic ring opening of dihydropyrimidines. Can catalyzes the ring opening of 5,6-dihydrouracil to N-carbamyl-alanine and of 5,6-dihydrothymine to N-carbamyl-amino isobutyrate.Tissue specificity:Liver and kidney.Involvement in disease:Defects in DPYS are the cause of dihydropyrimidinase deficiency (DHPD). DHPD is an autosomal recessive disorder characterized by dihydropyrimidinuria and associated with a variable clinical phenotype: epileptic or convulsive attacks, dysmorphic features and severe developmental delay, and congenital microvillous atrophy."