[Human transmissible spongiform encephalopathies].
[Human transmissible spongiform encephalopathies].
复制标题
[人类传染性海绵状脑病]。
DOI：
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发表时间：
2003
期刊：
影响因子：
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通讯作者：
E. Kahana
中科院分区：
文献类型：
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作者：
I. Leibovitz;D. Zamir;I. Polychuck;E. Kahana
1996年发现了克雅氏病的一种新变种。这种新变种被发现与脑病有关。已知有 5 种人类传染性海绵状脑病，全部由朊病毒引起。朊病毒是一种比病毒还要小的纯蛋白质，源自人体细胞壁的正常糖蛋白。这种蛋白质的功能尚不清楚，但推测与铜掺入超氧化物歧化酶有关。克雅氏病是一种早期痴呆症，伴有神经元损失、淀粉样蛋白积累和大脑海绵状变性。我们讨论克雅氏病及其变种的流行病学、诊断和治疗。预期寿命的增加肯定会导致这些疾病更加普遍，从而对发展中国家和发达国家的经济产生一定的影响。未来的治疗将集中于防止病理性朊病毒的产生或提取细胞内朊病毒。
A new variant of Creutzfeldt-Jakob disease was discovered in 1996. This new variant was found to be related to encephalopathy. There are 5 known human transmissible spongiform encephalopathies, all created by prions. The prion, a pure protein, smaller than a virus is derived from a normal glycoprotein of human cells wall. The function of this protein is unknown but supposed to be connected with copper incorporation into superoxide dismutase. Creutzfeldt-Jakob disease is an early dementive disease with loss of neurons, accumulation of amyloid and spongiform degeneration of the brain. We discuss the epidemiology, diagnosis and therapy of Creutzfeldt-Jakob and its variant. The increased life expectancy will certainly cause these diseases to be more common, thereby having a definite affect on the economy in both developing and developed countries. Future therapy will concentrate on either preventing the creation of pathologic prions or extracting intracellular prions.