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A prion / ˈ p r iː ɒ n / ⓘ is a misfolded protein that induces misfolding in normal variants of the same protein, leading to cellular death. Prions are responsible for prion diseases, known as transmissible spongiform encephalopathy (TSEs), which are fatal and transmissible neurodegenerative diseases affecting both humans and animals.
The subsequent demonstration that human prion diseases were transmissible reinforced the importance of spongiform change as a diagnostic feature, reflected in the use of the term "spongiform encephalopathy" for this group of disorders. Prions appear to be most infectious when in direct contact with affected tissues.
Prions are proteins of particular amino acid sequences in particular conformations. They propagate themselves in host cells by making conformational changes in other molecules of protein with the same amino acid sequence, but with a different conformation that is functionally important or detrimental to the organism.
The abnormal protein PrP Sc accumulates in the brain and destroys nerve cells, which leads to the mental and behavioral features of prion diseases. [citation needed] Several other changes in the PRNP gene (called polymorphisms) do not cause prion diseases but may affect a person's risk of developing these diseases or alter the course of the ...
Self-replication is a fundamental feature of life. It was proposed that self-replication emerged in the evolution of life when a molecule similar to a double-stranded polynucleotide (possibly like RNA) dissociated into single-stranded polynucleotides and each of these acted as a template for synthesis of a complementary strand producing two double stranded copies. [4]
The prion-rich bead fraction is subsequently harvested and tested. [10] Commonly tested tissues are brain homogenates and lymph tissues; however, prions have also been detected in skin and blood samples. [11] [12] [13] Certain tissues can be difficult to test for prions. For example, blood samples tend to have low levels of circulating ...
Reproducibility, closely related to replicability and repeatability, is a major principle underpinning the scientific method.For the findings of a study to be reproducible means that results obtained by an experiment or an observational study or in a statistical analysis of a data set should be achieved again with a high degree of reliability when the study is replicated.
The basic reproduction number of an infection is the expected number of subsequent cases it is likely to cause through transmission. [ 9 ] Virulence involves pathogens extracting host nutrients for their survival, evading host immune systems by producing microbial toxins and causing immunosuppression .