Question

Mention a disease caused by a prion.

Answer 1

Prion:

1. A prion is a kind of protein that triggers the normal proteins in the brain to fold abnormally.
2. Example: BSE (bovine spongiform encephalopathy) and Gerstmann-Straussler-Scheinker Syndrome.

Structure of Prions:

1. Prions are found all over the body but the ones that cause diseases are structurally different.
2. Few of them are even resistant to proteases.
3. The two isoforms of prions are:
4. PrPc: These prion proteins are found on the cell membrane and play an important role in cell signaling and cell adhesion.
5. More research is being carried out to discover its functions.
6. PrPsc: This is the disease-causing prion and is resistant to proteases. It affects the confirmation of PrPc and changes it. They are believed to have more beta sheets than the alpha helices.
7. It also forms highly structured amyloid fibers.

Types of Prion diseases:

1. The three main forms are: genetic, sporadic, and acquired.
2. Genetic disease is caused by an abnormality in a gene (specifically a mutation in the prion protein gene, PRNP).
3. Sporadic disease is of unknown cause.
4. Acquired disease results from the transmission of disease from an animal or another human disease.

Causes of prion diseases:

1. These are associated with misfolded proteins in the central nervous system.
2. And research has found that some of these misfolded proteins may be prions.

Prion diseases:

BSE (bovine spongiform encephalopathy):

1. It is a progressive neurological disorder of cattle that results from infection by an unusual transmissible agent called a prion.
2. The nature of the transmissible agent is not well understood.

Gerstmann-Straussler-Scheinker Syndrome:

1. Gerstmann-Sträussler-Scheinker disease is a prion disease that causes loss of coordination followed by the slow deterioration of mental function.
2. The disease is fatal, usually in about 5 years.