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Prions

What is Prions?

The term prion refers to infectious particles containing cells. Prions are infectious agents responsible for several neurodegenerative diseases in mammals, e.g., Creutzfeldt Jacob disease. This is due to the abnormal folding of proteins in the brain.

prions structure

It refers to the idea that the only infectious agents are proteins. He explained why coatings are resistant to UV rays. They are able to break down nucleic acids, but they trap metabolites.

Structure of Prions

Prions are found throughout the body but pathogens are structurally diverse. Few of them are even resistant to proteases. There are two isoforms of the prion:

These prion proteins are located on the cell membrane and play important roles in cell signaling and cell adhesion. Further studies are being conducted to investigate its functions.

This is a prion and protease inhibitor. It affects the validation of PrPc and reverses it. They are thought to contain more beta sheets than alpha helices.

It also forms highly structured amyloid fibrils. Other free proteins also attach to the ends of these filaments. Similar prions with the same amino acids can only bind. However, cross-species connection is also possible, but rare.

Caused by Prions

Prions are very rare and difficult to spread. However, they are progressive neurodegenerative diseases for which there is no treatment or cure. These diseases develop slowly.

These proteins affect animals other than humans. such as scrapie in sheep, mad cow disease in cattle, chronic disseminated disease in heifers. Prion diseases in humans include Creutzfeldt-Jacob disease, fatal familial insomnia, and Kuru disease.

Prions can spread in the human brain for years without symptoms. Prions start killing neurons and the signals hit the brain in an instant. Soon the man’s health begins to deteriorate.

All prion diseases are fatal, some lasting several months, some last years. Limited experimental evidence indicates that prions are not conventional infectious agents. It is considered a “self-replicating protein”.

Types of Prion Diseases

  • Prion diseases are caused by infection with infectious cells. Although dangerous, humans rarely catch these prions. For example, prions in leprosy were transmitted to humans through cannibalism. The main source was the New Guinea pig.
  • Familial prion diseases occur as a result of gene transmission. However, he is not an ancestral descendant. It can be caused by certain DNA mutations.
  • Prion diseases are also thought to occur periodically. This means the cause is uncertain. To date, such prion infections are the most common.
  • Abnormal folding and accumulation of prions in the brain leads to brain damage. This can lead to memory loss, personality changes and mobility problems.
  • Prions are the most dangerous infections caused by factors already present in the body and are often fatal. But not much has been discovered about prion diseases.

 

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