Prions are a special class of infectious agents that represent proteins with abnormal structure.
Prions are a special class of infectious agents that represent proteins with abnormal structure. They are able to catalyze the transformation of a normal membrane protein PrP into prions. This will start a chain reaction in which a large number of abnormal molecules. The last contact with each other forming amyloid — growing protein aggregates that accumulate in tissues and cause her damage. With the disease of Creutzfeldt — Jakob disease (spongy entsefalita) destroys the cerebral cortex and other parts of the brain that inevitably leads to death of the patient.
Scientists have learned to synthesize prions, affecting the organism of rodents, but, as shown by experiments with humanized animals (embedded in human genes), these proteins are not able to infect humans. In the new work, the researchers were able to create a pathogenic human prion protein from genetically modified PrP DNA that was introduced into the bacterium Escherichia coli. Biologists also found that the ganglioside GM1 protein is involved in the regeneration of nerve tissue, contributes to the reproduction of prions and the transmission of the disease from one person to another.
In addition, it was found that the speed of replication of prions, infectivity and vulnerability of certain brain structures is not determined by the presence of incorrectly folded proteins as such, but modifications in the structure of the molecule, particularly the C-terminal domain fragment located at one end of the connection.
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