Prions, prionoids and pathogenic proteins in Alzheimer disease

被引:79
作者
Ashe, Karen H. [1 ,2 ,3 ]
Aguzzi, Adriano [4 ]
机构
[1] Univ Minnesota, Dept Neurol, N Bud Grossman Ctr Memory Res & Care, Minneapolis, MN 55455 USA
[2] Univ Minnesota, Dept Neurosci, N Bud Grossman Ctr Memory Res & Care, Minneapolis, MN 55455 USA
[3] VA Med Ctr, GRECC, Minneapolis, MN USA
[4] Univ Zurich Hosp, Inst Neuropathol, CH-8091 Zurich, Switzerland
关键词
prions; prionoids; PrP; amyloid-beta; tau; pathogenic proteins; Alzheimer disease; NEUROFIBRILLARY TANGLES; TRANSGENIC MICE; NEURONAL LOSS; MOUSE MODEL; CAG REPEAT; TRANSMISSION; EXPANSION; PATHOLOGY; TOXICITY; SCRAPIE;
D O I
10.4161/pri.23061
中图分类号
Q5 [生物化学]; Q7 [分子生物学];
学科分类号
070307 [化学生物学]; 071010 [生物化学与分子生物学];
摘要
Like patients with prion disease, Alzheimer patients suffer from a fatal, progressive form of dementia. There is growing evidence that amyloid-beta (A beta) aggregates may be transmissible similar to prions, at least under extreme experimental conditions. However, unlike mice infected with prion protein (PrP) prions, those inoculated with A beta do not die. The transmission of A beta and PrP thus differs conspicuously in the neurological effects they induce in their hosts, the difference being no less than a matter of life and death. Far from being a mere academic nuance, this distinction between A beta and PrP begs the crucial questions of what, exactly, controls prion toxicity and how prion toxicity relates to prion infectivity.
引用
收藏
页码:55 / 59
页数:5
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