Prion-associated diseases are also known as transmissible spongiform encephalopathies, or TSEs. TSEs of humans include kuru, Creutzfeldt–Jakob disease, Gerstmann–Sträussler–Scheinker syndrome, fatal familial insomnia, variably protease-sensitive prionopathy, and familial spongiform encephalopathy. Unlike other infections, the transmissible material for all TSEs is aberrantly folded proteins. As a group, these disorders cause progressive impairment of central nervous system function that manifests as memory loss, personality changes, and/or abnormal movements. TSEs are progressive, incurable, and ultimately fatal. The term “spongiform encephalopathy” is derived from the classic post-mortem histologic findings of tiny, sponge-like holes found throughout the cerebral cortex in the brains of afflicted individuals.

错误:搜索内容不能为空,请输入英文关键词
错误:关键词超出字数限制,请精简
高级检索

Prion Associated Diseases

  • Joseph B. Domachowske

摘要

Prion-associated diseases are also known as transmissible spongiform encephalopathies, or TSEs. TSEs of humans include kuru, Creutzfeldt–Jakob disease, Gerstmann–Sträussler–Scheinker syndrome, fatal familial insomnia, variably protease-sensitive prionopathy, and familial spongiform encephalopathy. Unlike other infections, the transmissible material for all TSEs is aberrantly folded proteins. As a group, these disorders cause progressive impairment of central nervous system function that manifests as memory loss, personality changes, and/or abnormal movements. TSEs are progressive, incurable, and ultimately fatal. The term “spongiform encephalopathy” is derived from the classic post-mortem histologic findings of tiny, sponge-like holes found throughout the cerebral cortex in the brains of afflicted individuals.