CAJ | 학술논문

羊瘙痒病是累及山羊及绵羊的可传播海绵状脑病。为了观察羊瘙痒因子（Scrapie）的病原特征及病理组织改变特点，将羊瘙痒因子263K毒株颅内接种至金黄地鼠。经过81～110天的潜伏期，89%的动物发病（17／19只）。对发病地鼠的神经病理学检测发现，海绵状空泡变性的检出率为59%，淀粉样斑的检出率为17.6%。利用免疫组化和蛋白酶消化后的Westernblotting检测证实，100%的发病地鼠的脑组织中都出现蛋白酶抗性朊蛋白（PrP-res）。17只发病地鼠脑组织提取物中，PrP-res的泳动位置和分子量大小完全一致，出现两条分子量在25kD～31kD的反应带。尝试应用快速玻片印迹法检测病变组织中的PrP-res，结果显示，与常规固定包埋切片的免疫组化检出效果相似。这提示脑组织印片法可成为临床检测克-雅氏病（Creutzfeldt-Jacobdisease，CJD）患者脑组织活检标本中PrP-res的快速、有效的方法。羊瘙痒因子263K成功感染金黄地鼠再次证明，金黄地鼠是TSE感染因子良好的动物模型，发病率高，潜伏期短，发病动物PrP-res的检出率明显高于典型病理改变的检出率。新生成的PrP-res的电泳类型与接种的TSE因子有关，与宿主的个体差异无关，提示TSE感染因子的确存在“株”的现象。
양소양병시루급산양급면양적가전파해면상뇌병。위료관찰양소양인자（Scrapie）적병원특정급병리조직개변특점，장양소양인자263K독주로내접충지금황지서。경과81～110천적잠복기，89%적동물발병（17／19지）。대발병지서적신경병이학검측발현，해면상공포변성적검출솔위59%，정분양반적검출솔위17.6%。이용면역조화화단백매소화후적Westernblotting검측증실，100%적발병지서적뇌조직중도출현단백매항성원단백（PrP-res）。17지발병지서뇌조직제취물중，PrP-res적영동위치화분자량대소완전일치，출현량조분자량재25kD～31kD적반응대。상시응용쾌속파편인적법검측병변조직중적PrP-res，결과현시，여상규고정포매절편적면역조화검출효과상사。저제시뇌조직인편법가성위림상검측극-아씨병（Creutzfeldt-Jacobdisease，CJD）환자뇌조직활검표본중PrP-res적쾌속、유효적방법。양소양인자263K성공감염금황지서재차증명，금황지서시TSE감염인자량호적동물모형，발병솔고，잠복기단，발병동물PrP-res적검출솔명현고우전형병리개변적검출솔。신생성적PrP-res적전영류형여접충적TSE인자유관，여숙주적개체차이무관，제시TSE감염인자적학존재“주”적현상。
Scrapie is a kind of transmissible spongiformencephalopathies(TSE)involving in sheep and goat. To study the characteristics of the infectious agent and the neuropathology, Scrapies agent 263K was inoculated into the brains of the hamsters. 81 to 110 days after inoculation, 89% animals(17/19)appeared typical symptoms of TSE. Neuropathological studies revealed that 59% infected hamsters showed spongiform degeneration and 17% showed amyloid plaques. By immunohistochemistry and proteinase K-digested Western blot, the proteinase-resistant PrP-res proteins were detected in the brain tissues from all the infected animals. The electrophoresis patterns and the evaluated molecular weights of the PrP-res proteins from 17 infected hamsters were all the same, presenting two bands between roughly 25 kD and 31 kD in Western blot assays. Moreover, imprints of fresh brain tissues on slides were tested for PrP-res proteins. It showed similar results as that of routinely used paraffin-embedded slides by immunohistochemistry assays, which implies that this technique might be an effective method to detect PrP-res in the brain biopsies of the patients with CJD. Successful infection of Scrapie agent 263K in hamsters confirmed that baby hamster is an ideal model for TSE agents, with shorter incubation period and higher morbidity. The detecting rate of PrP-res is clearly higher than that of the neuropathological changes in the brain tissues from the infected animals. The electrophoresis patterns of the newly formed PrP-res proteins depend on the inoculated TSE agents, but not the individual animals, suggesting that there might be various “strains" among TSE agents.