In 1957, American researcher Daniel Carleton Gajdusek joined District Medical Officer Vincent Zigas in Papua New Guinea to investigate a strange illness. Among the Fore people, a fatal brain disease was killing nearly two percent of the population annually, mostly women and children. They called it kuru, meaning ‘shivering’. The disease caused victims to lose muscle control and suffer from uncontrollable bursts of laughter, leading outsiders to call it the ‘laughing death’. Gajdusek suspected a genetic cause but found no sign of an immune response. The transmission was eventually linked to mortuary rites where relatives consumed the deceased's brain. The Fore had stopped this practice by 1960 following government and missionary pressure, but the infectious agent remained a mystery.
The Discovery of Prions and Creutzfeldt-Jakob Disease
How scientists discovered prions, misfolded proteins with no genes that cause kuru, mad cow disease and Creutzfeldt-Jakob disease.
The Laughing Death
Frequently Asked Questions
What is a prion?
A prion is an infectious agent made only of protein. A misfolded prion can cause normal proteins to change shape, leading to a chain reaction that destroys brain cells. Unlike viruses or bacteria, it contains no genetic material like DNA or RNA.
What was Kuru or 'the laughing death'?
Kuru was a fatal brain disease found among the Fore people of Papua New Guinea. It caused trembling and uncontrollable bursts of laughter. Researchers discovered it was spread through mortuary rites where relatives consumed parts of the deceased, including the brain.
Can prion diseases spread between different species?
Yes. The 'Mad Cow Disease' crisis in the 1990s showed that a prion disease in cattle, called BSE, could infect humans who ate contaminated meat. This caused a new human disease known as variant Creutzfeldt-Jakob disease, or vCJD.
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