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病原生物学教研室 Department of pathogenic Biology of Gannan Medical University

医学微生物学 Medical Microbiology. 病原生物学教研室 Department of pathogenic Biology of Gannan Medical University. 第 27 章 朊粒 ( Prion ). 本质 :传染性蛋白因子,主要成分是一种 蛋白酶抗性蛋白( PrP ) 生物学特性 : 可滤过性,增殖非常缓慢; 与已知病毒特征明显不同; 不具有病毒结构,未检出核酸; 对福尔马林、蛋白酶、加热、电离辐射、紫外 线抵抗力强;. β 折叠. PrP C (正常).

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病原生物学教研室 Department of pathogenic Biology of Gannan Medical University

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  1. 医学微生物学 Medical Microbiology 病原生物学教研室 Department of pathogenic Biology of Gannan Medical University

  2. 第27章 朊粒(Prion) • 本质:传染性蛋白因子,主要成分是一种 蛋白酶抗性蛋白(PrP) • 生物学特性: 可滤过性,增殖非常缓慢; 与已知病毒特征明显不同; 不具有病毒结构,未检出核酸; 对福尔马林、蛋白酶、加热、电离辐射、紫外 线抵抗力强;

  3. β折叠 PrPC(正常) Prion-羊瘙痒病PrPC与PrPSC的三维结构 PrPSC(致病)

  4. 致病特点 • 是一种慢性、进行性、退化性病变为特征的、致死性CNS疾病  潜伏期长  发病呈慢性、进行性发展以死告终  病变以脑神皮质神经元空泡变性,并有   淀粉样斑块形成  不能诱导产生特异性免疫应答  以痴呆、共济失调、震颤为主要表现

  5. 致病 • 震颤病或库鲁(Kuru)病 • 克-雅病(CJD)或克-雅病变种(Variant CJD) • Gerstmann-Straussler综合征 • 致死性家族失眠症 • 羊瘙痒病 • 牛海绵脑病(疯牛病) • 传染性雪貂白质脑病 • 大耳鹿慢性消耗病

  6. 微生物学检查 • 免疫组化 • WB • 基因分析 • 血浆酶原沉淀

  7. 防治原则 • 无疫苗、无有效药物 • 预防:医源性预防、BSE及CJD预防

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