Research involving a former brain-eating tribe from Papua New Guinea is helping scientists better understand mad cow disease and other so-called prion conditions and may also offer insights into Parkinson’s and dementia.
People of the Fore tribe, studied by scientists from Britain and Papua New Guinea, have developed genetic resistance to a mad cow-like disease called kuru, which was spread mostly by the now abandoned ritual of eating relatives’ brains at funerals.
Experts say the cannibalistic practice led to a major epidemic of kuru prion disease among the Fore people, which at its height in the late 1950s caused the death of up to 2 percent of the population each year.
In findings published in the scientific journal Nature, the researchers said they had identified the specific prion resistance gene – and found that it also protects against all other forms of Creutzfeldt-Jakob disease (CJD).
Prions are infectious agents that cause often fatal brain diseases such as CJD in humans, scrapie in sheep and BSE (bovine spongiform encephalopathy, or mad cow disease) in cattle.
They are also a rare but important cause of dementia, and scientists say it is now recognised that the process involved in these diseases – in which prion proteins change shape and stick together to form polymers that damage the brain – is also what happens in common dementias such as Alzheimer’s, and in Parkinson’s and other neurodegenerative diseases.
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