Kuru

Kuru is a fatal prion disease that caused progressive neurological decline among the Fore people of Papua New Guinea. It spread through mortuary practices involving consumption of deceased relatives’ tissues.

Prion: A proteinaceous infectious agent that propagates by inducing misfolding of a normal host protein. Kuru is caused by infectious prions that accumulate in the nervous system.
Fore people: An Indigenous people of the Eastern Highlands of Papua New Guinea. Kuru became concentrated among the Fore and neighboring communities.
Michael Alpers: An Australian physician and researcher who conducted long-term studies of kuru in Papua New Guinea. His surveillance documented the epidemic’s decline and unusually long incubation periods.
Cannibalism: The consumption of human flesh by humans. The specific mortuary form of cannibalism associated with kuru enabled transmission.
PRNP: The gene that encodes the prion protein, whose altered forms cause inherited prion diseases and influence susceptibility. Variants in this gene helped some Fore people resist kuru.
Papua New Guinea Highlands: A mountainous region of Papua New Guinea inhabited by diverse Indigenous communities. The isolated valleys of the region shaped the disease’s local distribution.
Joseph Gibbs: An American neuropathologist who collaborated in research on kuru and other transmissible spongiform encephalopathies. His laboratory work supported the experimental transmission of kuru.
Human prion disease: A fatal neurological disorder caused by abnormal prion protein in humans. Kuru broadened recognition of acquired human prion diseases.
Prion propagation: The process by which misfolded prion protein converts normally folded prion protein into the same abnormal form. This self-amplifying conversion explains kuru’s long incubation and progressive course.
Mortuary feast: A funeral observance in which participants consume food as part of commemorating the dead. Cannibalistic mortuary practices transmitted kuru-infected tissue among kin.

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