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Scientists synthesize artificial strains of human prion

Penelitian - A team at Case Western Reserve University in Cleveland has created the first man-made prion. Scientists synthesize the first artificial human prions that provide a basis for understanding how subtle differences in protein structure and hope for new therapeutic strategies use analogous drugs with inhibitory or blocking effects on replication.

Human prions or proteins that have been folded incorrectly can lead to progressive fatal and progressive neurodegenerative diseases including the most common Creutzfeldt-Jakob disease. Molecules bind adjacent normal proteins in the brain to trigger a domino effect that causes microscopic opening and turns the brain into a sponge that results in progressive damage, dementia and death.

Penelitian Scientists synthesize artificial strains of human prion

"Until now our understanding of prions in the brain is very limited. Producing a synthetic human prion in test tubes will allow us to achieve a richer understanding of prion and replication structures," said Jiri Safar of the Department of Pathology at Case Western Reserve University.

Other researchers have made some form of prion lab-rodent, but not transmitted to humans as assessed in experiments with mouse models. New findings are becoming important for developing replication and propagation inhibitors throughout the brain that are essential for stopping prion-based brain disease.

Safar and colleagues synthesized a very destructive human prion from a genetically engineered human prion protein expressed in Escherichia coli. The team also found an important cofactor known as GM1 ganglioside is a cell molecule that modulates cell-to-cell signaling in triggering infection and transmission replication.

The speed of replication, infectivity, and targeting of specific brain structures by synthetic and natural prions is determined not by faulty prions, but by certain variations and modifications in the molecular structure especially those that control the growth rate of infectious prions. Misfolded protein structures affect transmissibility, cellular targeting and manifestations in humans.

Journal : Chae Kim et al. Artificial strain of human prions created in vitro, Nature Communications, 04 June 2018, DOI:10.1038/s41467-018-04584-z

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