神经退行性疾病中D-青霉胺的代谢：一项体内/体外巯基甲基化研究

D-penicillamine metabolism in neurodegenerative diseases: an in vivo/in vitro sulphydryl methylation study.

Peters L, Steventon G B, Green S, Sturman S, Waring R H, Williams A C

Faculty of Applied Sciences, University of the West of England, Bristol, UK.

Xenobiotica. 1994 Oct;24(10):1013-20. doi: 10.3109/00498259409043298.

Abstract

D-Penicillamine (125 mg) was administered orally to control (healthy volunteers), Parkinson's and Motor Neurone Disease patients following an overnight fast. 2. Blood was collected at 08:00 h for the preparation of red blood cell membranes used in the in vitro S-methylation studies. Urine was collected from 08:00 to 16:00 h and analysed for D-penicillamine and its metabolites. 3. Metabolism occurred via S-methylation, N-acetylation and disulphide formation. Both the Parkinson's and Motor Neurone Disease patients excreted significantly higher median levels of S-methyl-D-penicillamine in the urine than the controls (177 and 209% more for the Parkinson's and Motor Neurone Disease patients respectively). 4. The in vitro and in vivo production of S-methyl-D-pencillamine was highly correlated in the control (rs = 0.936), Parkinson's (0.986) and Motor Neurone Disease (0.752) populations.

摘要

禁食过夜后，对健康志愿者、帕金森病患者和运动神经元病患者口服给予D-青霉胺（125毫克）进行对照研究。2. 于08:00采集血液，用于制备体外S-甲基化研究中使用的红细胞膜。在08:00至16:00收集尿液，并分析其中的D-青霉胺及其代谢产物。3. 代谢通过S-甲基化、N-乙酰化和二硫键形成发生。帕金森病患者和运动神经元病患者尿液中S-甲基-D-青霉胺的排泄中位数水平均显著高于对照组（帕金森病患者和运动神经元病患者分别高出177%和209%）。4. 在对照组（rs = 0.936）、帕金森病组（0.986）和运动神经元病组（0.752）人群中，体外和体内S-甲基-D-青霉胺的生成高度相关。