Prion protein
Prions are infective agents composed of protein. Prion are made of proteins called PrP. PrP exists in a normal form (PrPC) and an abnormal form (PrPSc). PrPSc is resistant to protease and therefore cannot be broken down in the body. PrPSc can change adjacent PrPC into PRPSc (this is how the 'infection' spreads).
PrPC is a normal constituent of cell membranes and is an alpha-helical structure. In PrPSc much of the alpha-helical structure is replaced by a beta-sheet structure.
Prions cause disease by aggregating into plaques in the extracellular space of the central nervous system and therefore disrupt normal tissue structure.
PrPC is a normal constituent of cell membranes and is an alpha-helical structure. In PrPSc much of the alpha-helical structure is replaced by a beta-sheet structure.
Prions cause disease by aggregating into plaques in the extracellular space of the central nervous system and therefore disrupt normal tissue structure.
