A fast protein liquid chromatography system for purifying prion proteins

脑中错误折叠、聚集的蛋白质的存在是许多人类神经退行性疾病的标志。特别令人感兴趣的是朊病毒疾病，其中包括人类的克雅氏病（CJD）和牛的牛海绵状脑病（BSE，或“疯牛病”）。在这些疾病中，称为朊病毒的畸形蛋白质在大脑中积累，最终导致脑细胞死亡。朊病毒因其具有传染性而臭名昭著。通常，来自一个物种的朊病毒不会有效地感染其他物种，这一过程被称为“物种屏障”。有时，物种屏障是不完善的，例如在英国疯牛病流行期间BSE朊病毒向人类传播的情况。一些动物物种，如狗和马，似乎对朊病毒病有抵抗力。在光谱的另一端，银行田鼠（

The presence of misfolded, aggregated proteins in the brain is a hallmark of many human neurodegenerative diseases. Of particular interest are the prion diseases, which include Creutzfeldt-Jakob disease (CJD) in humans and bovine spongiform encephalopathy (BSE, or “mad cow disease”) in cattle. In these diseases, misshapen proteins called prions accumulate in the brain, eventually resulting in the death of brain cells. Prions are notorious for being infectious proteins. Generally, prions from one species do not efficiently infect other species, a process referred to as the “species barrier”. Sometimes, the species barrier is imperfect, as in the case of the transmission of BSE prions to humans during the mad cow disease epidemic in the United Kingdom. Some animal species, such as dogs and horses, are seemingly resistant to prion disease. At the other end of the spectrum, bank voles (