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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.
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.
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]
For example, the proton spin puzzle, the EMC effect, the distributions of electric charges inside the nucleons, as found by Robert Hofstadter in 1956, [2] [3] and the ad hoc CKM matrix elements. When the term "preon" was coined, it was primarily to explain the two families of spin- 1 / 2 fermions: quarks and leptons. More recent preon ...
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.
Others insert additional amino acids into the protein or cause an abnormally short protein to be made. These mutations cause the cell to make prion proteins with an abnormal structure. 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]
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[2] [3] Typically, the term pathogen is used to describe an infectious microorganism or agent, such as a virus, bacterium, protozoan, prion, viroid, or fungus. [4] [5] [6] Small animals, such as helminths and insects, can also cause or transmit disease. However, these animals are usually referred to as parasites rather than pathogens. [7]