Gene Mutation Provides Resistance to Prion Disease; A Cure for Cannibalism?
KJ Belonio | | Jun 17, 2015 05:10 PM EDT |
(Photo : Getty Images/Christopher Furlong) Scientists have recently discovered that a gene mutation showed complete resistance against prion-related diseases. The study was published in the journal Nature.
A new study recently revealed that a gene mutation showed complete resistance against a type of prion disease.
According to a team of researchers from UCL Institute of Neurology in the U.K. as well as researchers from New Guinea, the resistant gene mutations were discovered after they studied the genes of people believed to be resistant to Kuru, a type of prion disease.
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In the study published in the journal Nature, scientists described a genetic variant that appears to stop misfolded proteins known as prions from propagating in the brain. And through the help of some members of the Fore people of Papua New Guinea, who experienced an outbreak of a deadly brain-wasting disease half a century ago, experts were able to note a naturally occurring variant of a brain protein that prevents prion diseases such as Kuru and Mad Cow disease, Science News revealed.
"We've never seen anything before that is completely protective," study coauthor and University College London neurologist and molecular biologist John Collinge said. "It just switches off the disease."
In the previous research, scientists have discovered that some people seemed to be less susceptible to prion diseases if they have an amino-acid substitution in a particular region of the prion protein known as codon 129, Nature.com learned. But in the most recent analysis, another protective mutation, codon 127, has been found.
So, can the latest evolution finally devise a cure for prion diseases? Well, thanks to the efforts of researchers, there may be a way for people to prevent getting prion-related diseases.
In the late 1950s, the Fore people of Papua New Guinea were infected by the deadly kuru disease, which killed up to 2 percent of the group's population each year. And scientists later traced the illness to ritual cannibalism, in which members of the tribe ate the brains and nervous systems of their dead.
Scientists also noted that the outbreak most likely started when a Fore person ate the body parts from someone who had sporadic Creutzfeldt-Jakob disease (CJD), a type of prion disease that suddenly affects about one person in a million each year.
Meanwhile, the two genetic mutations discovered in the volunteers' DNA appeared to be linked to immunity from Kuru. According to Medical Xpress, researchers are planning to see if they can find out how or why the genetic mutation causes resistance.
The team also seek to determine if the same technique could be used to confer resistance in humans after the experiment made mice 100 percent resistant.
Tagscannibalism, Mad Cow Disease, Prions, Creutzfeldt Jakob Disease, cure, brain, Genetics
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