Creutzfeldt-Jakob disease

Resistance to prion disease in humans

Transmissible spongiform encephalopathies (TSEs) are rare human neurodegenerative disorders that are caused by infectious proteins called prions. A naturally occurring variant of the human prion has been found that completely protects against the disease. A protective variant of the prion protein was discovered in the Fore people of Papua New Guinea. Beginning in the early 1900s, the prion disease kuru spread among Fore women and children as …

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Detecting prions by quaking and shaking

The human prion disease, Creutzfeld-Jacob, is diagnosed by a variety of criteria, including clinical features, electroencephalograms, and magnetic resonance imaging. Until recently there was no non-invasive assay to detect PrPSc, the only specific marker for the disease. This challenge has been overcome using amplification procedures to detect Creutzfeldt-Jakob prions in nasal brushings and in urine. These …

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