由狂牛症淺談傳染性海綿狀腦病變及庫賈氏病

Mad Cow Disease, Transmissible Spongiform Encephalopathy, and Creutzfeldt-Jakob Disease

江佳錡(Chia-Chi Chiang) ； 陳建志(Chien-Chih Chen) ； 林光洋(Kuang-Yang Lin) ； 黃惠娟(Wai-Kuen Wong) ； 郭冠良(Kuan-Liang Kuo) ； 吳岱穎(Tai-Yin Wu)

《北市醫學雜誌》 3卷7期 (2006/07) Pp. 647-654

https://doi.org/10.6200/TCMJ.2006.3.7.01

摘要

在目前已知的感染性致病原中，普利旺蛋白質是唯一不含核酸的致病粒子，不同於一般的病毒或細菌，正常的普利旺蛋白質可經由傳染、基因遺傳、基因突變或自發性等途徑轉變成致病性的普利旺蛋白質，進而造成普利旺病。普利旺病又稱為傳染性海綿狀腦病變，因為患病動物的腦部會呈現海綿狀空洞的變化，臨床上出現失智、運動失調、失眠及行為異常等表現，一旦發病終將致死，其病程進展迅速及無有效治療方式，都是目前醫學的一大挑戰。我們所熟知的狂牛症、羊搔症、人類的庫賈氏病、致命性失眠症等，都是致病性普利旺蛋白質所造成。本篇文章即由狂牛症著手，淺談普利旺蛋白質、傳染性海綿狀腦病變、庫賈氏病。

關鍵字

普利旺蛋白質 ； 普利旺病 ； 傳染性海綿狀腦病變 ； 狂牛症 ； 庫賈氏病

並列摘要

Prion protein is one of the well-known infectious pathogens and the only pathogen which is devoid of nucleic acid. Not like other viral and bacterial infections, prion diseases result from the conversion of normal cellular prion protein to disease-causing isoform through various ways, such as infection, heredity, mutation, or spontaneous conversion. Prion disease is also named as transmissible spongiform encephalopathy because the spongiform degeneration is noted in the brain of infected animals. The clinical manifestations include dementia, ataxia, insomnia, deviant behavior, etc. The clinical course is progressive rapidly, and death always occurs in the terminal phase of the illness. There is no effective therapy for treating prion diseases in the present medical field. Not only the pathogenic mechanisms but also the curing courses have become the serious challenges and topics for the current medical science. From the recent studies of mad cow disease, scrapie, Creutzfeldt-Jakob disease (CJD), fatal insomnia diseases, etc., prion protein is confirmed as the pathogenic and important factor for transmissible spongiform encephalopathy. In this article, we will introduce mad cow disease, prion protein, transmissible spongiform encephalopathy, and Creutzfeldt-Jakob disease (CJD).

並列關鍵字

prions ； prion disease ； transmissible spongiform encephalopathy ； mad cow disease ； Creutzfeldt-Jakob disease