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精氨琥珀酸裂解酶(ASL)是尿素生成中第二种最易缺乏的酶。人体缺乏ASL将引起精氨琥珀酸尿,对10多例日本患者的分析发现,其ASL活性完全丧失,而且不含免疫交叉反应物质。但Simard等人报道,在美国发现的28例病人都可用蛋白印迹分析法检出交叉反应物质。1986年,O’Brien等人分离出人ASL cDNA克隆,从推导的氨基酸序列中发现,酵母和人氨基酸序列部分同源,Amaya等人报道了大鼠ASL cDNA克隆的核苷酸序列,发
Arginine succinate lyase (ASL) is the second most vulnerable enzyme in urea production. The absence of ASL in humans causes arginosuccinateuric acid. Analysis of more than 10 Japanese patients found that their ASL activity was completely absent and that they did not contain immune cross-reactive substances. However, Simard et al reported that 28 patients found in the United States were able to detect cross-reactive substances by Western blot analysis. In 1986, O’Brien et al. Isolated the human ASL cDNA clone. From the deduced amino acid sequence, yeast and human amino acid sequences were found to be partially homologous. Amaya et al. Reported the nucleotide sequence of the rat ASL cDNA clone,