You've prob heard of mad cow disease, but do you know what causes it to happen? The answer is prion. A prion is a type of protein that can cause disease in animals and humans by triggering normally healthy proteins in the brain to fold abnormally.
The mechanism of action for prions differs significantly from that of bacteria and viruses, as prions consist solely of proteins without any genetic material. When a misfolded prion enters a healthy individual, possibly through the consumption of contaminated food, it induces the conversion of properly folded proteins into the disease-associated conformation. The precise process by which this transformation occurs remains largely unknown.
In the brain of a cow affected by Bovine Spongiform Encephalopathy (BSE) or "mad cow" disease, this colored transmission electron micrograph (TEM) captures prion fibrils. Prions, akin to virus-like entities, consist of a prion protein. The elongated fibrils (green) are thought to be clusters of the protein responsible for the infectious prion. These prions target nerve cells, leading to neurodegenerative brain diseases. Symptoms of "mad cow" disease encompass glazed eyes and uncontrollable body tremors. Prions are responsible for BSE in cattle, scrapie in sheep and goats, and Creutzfeldt-Jakob disease in humans.
In conclusion, these misfolded proteins must be studied more in order to decipher these neurodegenerative diseases. Unraveling the secrets of prions holds the potential not only for advancing our comprehension of these peculiar proteins but also for developing targeted interventions to mitigate the impact of prion-related diseases.
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