Lebo R V, Martin D W
Biochem Genet. 1978 Oct;16(9-10):905-16. doi: 10.1007/BF00483742.
The product of the enzyme 5-phosphoribosyl-1-pyrophosphate (PPriboseP) synthetase is a substrate for purine, pyrimidine, and pyridine biosynthesis and may be rate limiting for purine biosynthesis. A system developed to electrophoretically separate and histochemically detect PPriboseP synthetase in crude cell extracts has facilitated the identification of electrophoretically variant enzyme forms in the erythrocytes of five patients from a patient population of 200. Additional studies of human organs obtained at autopsy revealed a unique electrophoretic mobility for nearly each organ within the same individual.
5-磷酸核糖-1-焦磷酸(PPriboseP)合成酶的产物是嘌呤、嘧啶和吡啶生物合成的底物,可能是嘌呤生物合成的限速物质。一个用于在粗细胞提取物中对PPriboseP合成酶进行电泳分离和组织化学检测的系统,已有助于在200名患者群体中的5名患者的红细胞中鉴定出电泳变异的酶形式。对尸检获得的人体器官的进一步研究显示,同一个体内几乎每个器官都有独特的电泳迁移率。