The Fore tribe of Papua New Guinea used to have a funeral religious rite in which mourner eat the brain of the gone . In the process , some were unintentionally infected by a mad cow – like prion disease called kuru . By the fifties , this drill was pop up to2 percentof the tribe every year . But then something awful — and yet wholly natural — happened : some Fore evolve a genetic resistance to the disease that remains in place today , even though the Fore have largely empty the brain - eat ritual .
harmonise to a new work published in the journalNature , the genes of this tribe might serve scientist unlock mysteries about neurodegenerative diseases , which could in turn of events lead to valuable insights on dementia .
Prions are infectious protein that guide to genius disease likeCreutzfeldt - Jakobdisease ( CJD ) , kuru , and fatal inherited insomnia in humans , scrapie in Capricorn the Goat and sheep , mad moo-cow disease in cattle , and chronic wasting disease in deer and elk .
premature research bump that people like the Fore with a course take place variant in the cistron that codes for the prion protein seemed to be protected from these diseases . In this new study , John Collinge and colleagues analyzed this variant prion protein , call G127V , to see how it affects susceptibleness to infective prion . What has the researchers intrigue is that this particular prion resistance factor also guards against all other forms of CJD .
On occasion , prionsalso make dementia . This matters for scientists who say that the process by which the prion disease take hold are the same as the processes that lead to Alzheimer ’s , Parkinson ’s , and other brain - attack diseases . Prions do this by essentially shape - shifting and then clumping together to work polymer that stimulate neurodegeneration .
Collingetold Reuters , “ This is a striking illustration of Darwinian evolution in humankind — the epidemic of prion disease select a exclusive familial change that provided pure protection against an invariably fateful dementia . ”
Collinge say the work will now be to sympathise the molecular basis of this effect , to examine more closely how misshapen proteins turn in the brain and lead to forms of dementia . In time , those findings could lead to new and better treatments of such disease .
[ h / tGuardian ]
