家族性肌萎缩侧索硬化症血中Cu/ZnSOD及MDA值变化与临床分型研究

THE ANALYSIS OF Cu/Zn SOD ACTIVITY AND MDA CONTENT IN BLOOD, CLASSIFIED STUDY ABOUT FAMILIAL AMYOTROPHIC LATERAL SCLEROSIS

目的 :探讨家族性肌萎缩侧索硬化症患者及家系成员铜锌超氧化物歧化酶 (Cu/ Zn SOD)活性和丙二醛 (MDA)含量变化及家族性肌萎缩侧索硬化症 (FAL S)的临床分型。方法 :采用生物化学方法测定红细胞内 Cu/ Zn SOD活性及 MDA值。结果 :在 2个家系中发现 6例 Cu/ Zn SOD活性下降 ,3例 MDA含量增高。结论 :有 Cu/ Zn SOD活性下降的 2个家系临床分型可能为肌萎缩侧索硬化症 AL S 1型 ,MDA含量增高间接反映 Cu/ Zn SOD变化。红细胞内 Cu/ Zn SOD活性测定及 MDA含量测定有可能作为 AL S1型患者及基因携带者的诊断方法之一 ,并可用于 FAL

Objective: To study Cu/Zn SOD activity and MDAcontent in blood of FALS and to study classification of FALS Methods:Biochemical methods were used to determine Cu/Zn SOD activity and MDA content Results: 6 members in the 2 familes had lower activity of Cu/Zn SOD 3 patients had higher content of MDA Conclusions: 2 families with degression of Cu/Zn SOD activity may be ALS 1 Rise in MDA content can reflect indirecty the Cu/Zn SOD enzyme activity The determination of Cu/Zn SOD and MDA can be used in diagnosis of ALS 1 and carriers of ALS 1 It can also be used in screening whether the mutation of Cu/Zn SOD genes occurrence and classification of FALS