8.3k views
3 votes
Where do PrP mutations often occur to lead to TSEs?

1 Answer

4 votes

Final answer:

PrP mutations that lead to TSEs, such as kuru and BSE, typically result from somatic mutations that change the normal prion protein into a misfolded infectious form, causing the characteristic brain tissue damage.

Step-by-step explanation:

PrP mutations, which lead to transmissible spongiform encephalopathies (TSEs) in humans and animals, often occur as somatic mutations that result in a single amino acid change. These mutations result in the refolding of the normal prion protein (PrPC) into the infectious form (PrPSC). This abnormal folding then leads to the accumulation of PrPSC, which can cause brain damage and the characteristic spongiform changes seen in these diseases.

Transmissible Spongiform Encephalopathies (TSEs) such as kuru, Creutzfeldt-Jakob disease, and Bovine Spongiform Encephalopathy (BSE) have been linked to both hereditary and acquired forms. Mutations in the germline PrP gene can lead to hereditary cases, while acquired cases may result from exposure to contaminated tissues through means such as consumption of infected meat, medical procedures with contaminated instruments, or organ transplants.

User BWA
by
8.6k points