The Prion Disease

10.30047/JGMB.199512.0001

Franklin M. C. Chen

prion ； protein infectious partcles ； spongiform encephalopathies ； mad cow diease ； protein folding

Journal of Genetics and Molecular Biology

6卷4期（1995 / 12 / 01）

51 - 53

英文

This paper reviews what is known about certain degenerative disorders of the central nervous system in animals and humans caused by protein prions (pronounced as preeons). An amino acid mutation in the protein results in an incorrectly folded protein conformation. It has been proposed that infection by the prion takes the form of the chaperon protein fold, forcing the healthy protein to assume an incorrectly folded conformation. However, several questions still remain. One issue is how the infected prion changes the conformation of the normal prion. Another issue is why the immune system in animals and humans cannot recognize and mount attacks to these alien proteins. The author addresses these issues in an attempt to evoke researchers’ attentions for better understanding the origin and the ramifications of the prion disease.

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