Final answer:
Mutation in the PrP gene can cause TSEs by producing abnormally folded prion proteins, leading to brain damage and diseases such as kuru and BSE. A somatic mutation in the PrP gene can lead to a single amino change, causing the refolding of the normal PrP protein into an infectious form.
Step-by-step explanation:
Another mutation in the PrP gene that can lead to Transmissible Spongiform Encephalopathies (TSEs) involves a somatic mutation leading to a single amino acid change. This results in the normal prion protein (PrPC) refolding into the infectious form, PrPSc. When PrPSc is introduced into the body, it binds to PrPC and converts it to PrPSc, causing an exponential increase of the abnormally folded prion protein. This aggregation is responsible for the sponge-like lesions observed in the brains of infected individuals or animals. Examples of TSEs in humans include kuru, fatal familial insomnia, Gerstmann-Straussler-Scheinker disease, and Creutzfeldt-Jakob disease. In animals, these include BSE (mad cow disease), scrapie, and chronic wasting disease. Transmission of TSEs can occur through various means including ingestion of infected meat, contaminated medical procedures, and certain hereditary factors.