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Mutated cannibals PDF Print E-mail
 A mutation in the prion protein protects against the deadly disease kuru. Experiments may help in the fight against mad cow disease. Members of the Fore tribe living in Papua New Guinea for many years were known for its controversial funeral rituals.

 To show respect for the dead, they eat their brains. In the fifties of last century, died of unknown causes about 2500 members of the tribe. Tests done a few years later showed that the cause of the deadly disease has its origins in this very ritual. Today known as the Kuru disease belongs to the group of neurodegenerative diseases caused by prions. Recently it was about them aloud on the occasion of mad cow disease so far responsible for the deaths of 166 people in the UK. Prions are small protein molecules, which can change in an infectious form of other, non-prion proteins in the body, leading to drastic changes in the structure of the brain by doctors described as spongy degeneration of the body. British researcher Simon Mead decided to once again look at Kuru victims. Using modern methods of molecular biology has analyzed the genetic sequence coding for prion protein in the dead persons and compared it to a sequence of still living members of the Fore tribe. In the comparison group were those who participated in the ritual of eating the brain before it was banned and those who have never participated in this practice. To his surprise he found that in 51 people who survived the ritual and in their offspring, a gene responsible for the formation of prion proteins differ in sequence from the genes of deceased persons. Well, exactly codon 127 of the prion protein gene, which in all previously known cases of encoding the amino acid glycine. For people resistant to prion in this place is glycine instead of valine. This single mutation causes the infectious prion proteins are not able to change those already present and the disease does not develop. A more detailed genetic analysis showed that the saving mutation appeared in a single person about 200 years ago. All their descendants have a mutated form of the prion protein protection from Kuru. This is a very striking proof of the theory of evolution and its main thesis, which says that the greater the chances of survival are people better suited to changing environmental conditions, and the driver of these changes are adaptive mutations. These findings could help to develop effective methods of prevention and treatment of other prion diseases occurring in humans. Currently, researchers examine whether the same mutation, which protected the part of the Fore tribe may prevent the development of mad cow disease and its human equivalent Creutzfeldt-Jakob disease.

 
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