historyNLM Circulating Now1950s · Papua New Guinea

D. Carleton Gajdusek and Kuru in New Guinea

the gist

Daniel Carleton Gajdusek, an American virologist, studied kuru among the Fore people of the South Fore region of Papua New Guinea in the 1950s. Kuru was a fatal degenerative brain disease that spread through the community, and it was linked to the custom of eating the dead's brains during funeral rites. Gajdusek showed that the disease could be transmitted, which meant it was caused by an unusual agent that could persist for years before symptoms appeared. Neurologist Stanley Prusiner later identified that agent as the prion, a misfolded protein, and Gajdusek shared the 1976 Nobel Prize in Physiology or Medicine for this work.

how it works

Prions are misfolded versions of a normal brain protein. When a misfolded form enters the brain, it causes normal proteins to fold the same wrong way, creating clumps that damage nerve cells. Because prions contain no DNA or RNA, they resist the usual methods used to destroy viruses and bacteria.

so what

Kuru showed that an infectious agent could be a protein with no genetic material, a discovery that overturned basic assumptions in biology. The same family of diseases includes Creutzfeldt-Jakob disease and bovine spongiform encephalopathy, known as mad cow disease. Gajdusek's own later conviction on child abuse charges is a reminder that his work is also controversial.

the bigger pattern

Prion diseases were a new category of illness that showed proteins alone could cause infection, which was later found in scrapie in sheep and in mad cow disease.

U.S. National Library of Medicine

Illustrative photo · Liberski PP, CC BY 3.0, via Wikimedia Commons