先天性红细胞生成性卟啉病和迟发性皮肤卟啉病患者酶活性的研究  

Enzyme Activity in Congenital Erythropoietic Porphyrias and Porphyria Cutanea Tardas.

作　　者：李存保[1] 王美玲 王文礼[1] 孟峻[1] 高建萍[1] 郭晓宇[1] 

机构地区：[1]内蒙古医学院,呼和浩特市010059

出　　处：《中国皮肤性病学杂志》1999年第5期263-264,共2页The Chinese Journal of Dermatovenereology

摘　　要：目的　研究和比较先天性红细胞生成性卟啉病（ Ｃ Ｅ Ｐ） 患者、迟发性皮肤卟啉病（ Ｐ Ｃ Ｔ） 患者和正常健康者的红细胞酶活性。方法　荧光分光光度法和反相高效液相色谱法。结果　 Ｃ Ｅ Ｐ 患者的红细胞尿卟啉原Ⅲ同合成酶（ Ｕ Ｒ Ｏ－ Ｃ Ｏ Ｓ） 的活性仅为正常健康者的１７ ％ ，而 Ｐ Ｃ Ｔ 患者的尿卟啉原Ⅰ脱羧酶（ Ｕ Ｒ Ｏ Ｄ） 活性仅为健康正常者的４９ ％ 。结论　 Ｕ Ｒ Ｏ－ Ｃ Ｏ Ｓ 和 Ｕ Ｒ Ｏ Ｄ 分别是引起 Ｃ Ｅ Ｐ 和 Ｐ Ｃ Ｔ 的主要损害部位。Objective To study and compare the enzyme activities of erythrocyte in Congenital erythropoietic porphyrias(CEP),Porphyria cutanea tardas(PCT) and healthy normals subjects.Methods The fluorospectrophotometry and reverse phase high performance liquid chromagraphy(RP HPLC).Results The erythrocyte URO COS activity of the CEP patients was lower than that of the normal healthy subjects,only account for 17% of the normal healthy subjects,and the UROD activty of PCT patients was 49% of the normal healthy subjects.Conclusion The CEP and PCT were caused by the deficiency of URO COS and UROD respectively.

关 键 词：卟啉病 红细胞 酶活性 皮肤卟啉病 

分 类 号：R589.802[医药卫生—内分泌]