ns\' 5. A 74-y/o lifetime xenophobic, agoraphobic, life-long vegetarian had been
ID: 210796 • Letter: N
Question
ns' 5. A 74-y/o lifetime xenophobic, agoraphobic, life-long vegetarian had been experiencing increasing dementia during the last few years. As a germaphobe she literally lives in a bubble by herself. She feared infectious diseases so much that all of her food was grown in her protected greenhouse free of all infectious particles. Her brain biopsy showed aggregations of certain proteins and hollow areas within the brain, but PCR and RT-PCR came back negative for pathogen DNA/RNA. (a) What was the agent that killed her? (b) What is the whole disease name? (c) How was it "contracted"? (d) What molecular test could be done to verify its identity? (e) What related disease has been found in cattle, particularly in the U.K. (use the medical term, not the media's name for it)?Explanation / Answer
a. The agent that killed her should be prions. Prions are infectious agents composed entirely of a protein material that can fold in multiple, structurally abstract ways, leading to disease in a manner that is epidemiologically comparable to the spread of viral infection.
b. The whole disease name is "Transmissible spongiform encephalopathy"
c. Since the patient had no contact with infectious particles, the prions should have been triggered through inherited germline mutations in the human prion protein gene.
d. Molecular diagnostic tests like PCR can be carried out to find out the mutations in the gene responsible for the production of human prion protein.
e. Bovine spongiform encephalopathy, which is also a prion disease, affected cattle in U.K.
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