Gene Found In Cannibalistic Tribe Can Prevent Human Mad Cow Disease

Researchers in London have identified a variant of the human prion protein that offers resistance to fatal diseases like the Creutzfeldt-Jakob disease.

Disease like Creutzfeldt-Jakob disease (CJD), are called prion diseases, the equivalent of mad cow disease in cattle. CJD is invariably fatal and can , be inherited, acquired from an infected person's tissue or bloodand from eating infected cattle. Though rare, CJD is a neurodegenerative disease without a cure. Prions are misfolded proteins that have infectious properties.

Researchers at University College London (UCL) examined members of the Fore community in Papua New Guinea who survived Kuru, a disease similar to CJD. Kuru was rampant in the community until late 1950s when members of the tribe stopped practicing cannibalism. Consumption of infected human tissue was blamed for the persistence of the disease in the community. Some members who were exposed to the disease but did not develop it are believed to have developed resistance to it.

UCL scientists who discovered a prion protein gene in survivors that protected against Kuru. They then hypothesized that gene that offers resistance to Kuru could also help develop resistance to other prion diseases like CJD. When genetically engineered mice with the protective gene were bred and tested in the lab, they showed resistance to prion diseases including the human variant of CJD.

"From the human genetic work the Unit has carried out in Papua New Guinea we were expecting the mice to show some resistance to disease. However, we were surprised that the mice were completely protected from all human prion strains. The result could not have been clearer or more dramatic," said Dr Emmanuel Asante, who led the study published in Nature.