Cannibalistic Tribe Develops Resistance to Fatal Disease

The practice of cannibalism in one Papua New Guinea tribe lead to the spread of a fatal brain disease called kuru that caused a devastating epidemic in the group. But now, some members of the tribe carry a gene that appears to protect against kuru, as well as other so-called “prion diseases,” such as mad cow, a new study finds.

 

The findings could help researchers better understand these fatal brain diseases, and develop treatments for people who have the diseases, the researchers said.

The Papua New Guinea tribe, known as the Fore people, used to conduct a funeral ritual that involved consuming the human brain.

 

Early in the 20th century, tribe members began to develop kuru, a neurological disorder caused by infectious prions, which are proteins that fold abnormally and form lesions in the brain. This was the start of an epidemic of kuru among the Fore people, which at its peak in the 1950s, killed up to 2 percent of the tribe each year.

 

The tribe stopped practicing cannibalism in the late 1950s, which lead to a decline in kuru. But because the disease can take many years to show up, cases continued to appear for decades.

 

Recently, researchers discovered that some of the people who survived the kuru epidemic carry a genetic mutation called V127, whereas those who developed kuru did not have this mutation. This led the researchers to suspect that V127 conferred protection against the disease.

 

In a new study, researchers genetically engineered mice to have the V127 mutation, and then injected the animals with infectious prions. Results showed that mice with one copy of the 127V mutation were resistant to kuru, as well as a similar disease called classical Creutzfeldt-Jakob disease. Mice with two copies of V127 were resistant to those diseases, as well as another prion disease, called variant Creutzfeldt-Jakob disease, which is sometimes referred to as the “human form of mad cow disease.”