What are Prions and their role?
Prions are the proteins that cause Mad Cow Disease also known as BSE. Prions are an infectious protein. Prions are defined as an proteinaceous infectious particle, the protein is capable of changing the conformation of proteins to match its own and becomes infectious without the use of genetic materials. They can cross between species such as cattle, and sheep. Extreme heats chemical agents can inactivate the proteins. Prions are naturally occurring in the body and are not considered foreign nor do they stimulate the immune system and will not hurt your body, however the disease causing prions contact the normal prions by physical contact and alter the normal prions therefore causing the disease. With Mad Cow Disease The Prions target the specific protein in the cell that is normal called PrPC
The role of protein Misfolding in BSE
Diseased proteins result from improper folding and therefore the misfolded proteins induces copies of the same protein to also again misfold and increases the disease progress when it is misfolded.
The scrapie prion PrPsc contacts and bumps into the normal cellular form PrPc intermediate and shifts the folding process converting them to the misfolded prion (the sheep version of mad cow disease called the PrPsc prion). The process continues over and over again. The body continues producing the normal cell PrPc and the scrapie prion PrPsc continues creating more replications of itself without needing any nucleic acid of its own.
The role of protein Aggregation in BSE
Proteosomes are known as the protein destroyer and therefore gets rid of non needed proteins, they clump together inside the cell which is called aggregation.
It continues building and then kills the cells causing the spongy appearance of the tissue which are the dying cells and continues the spread of the disease. Protein aggregation damage the nerve cells in mad cow disease and is in constant production.
The role of Chaperone Protein in BSE
As a chaperone the primary function is to prevent aggregation of the protein particles prions and therefore transmit and cause the infectious disease. Prions are a pure protein also known as an infectious agent which is self replication. The pure protein can be replicated itself by the pathological chaperone and the protein serves as its own chaperone.
The PrPsc version is hydrophobic and does not like water and as the PrPsc misfolded prions comes into contact with the normal PrPc and is converted to PrPSc- the misfolding continues to spread as the PrPsc uses itself as a chaperone to help the fold properly, once the chaperone PrPsc is converted it CAN NOT be converted back to the normal PrPC. As it continues to replicated itself the PrPsc cannot reverse to PrPc