Episode 16: The Neuroscience of Mad Cow Disease

Neuroscience: Amateur Hour

When I was a kid, I thought two things were going to be much bigger problems in my life than they actually are - quicksand and mad cow disease.
Turns out that they aren't but mad cow disease is still the stuff of science-fiction nightmares. Mad cow disease is caused by the spread of prions, proteins that weaponize our own cells against us and cause proteins to misfold and aggregate, slowly killing neurons throughout the brain. The disease is characterized by rapid and fatal neurodegeneration.
Want to get the shit scared out of you by learning about the truth behind mad cow disease? Come and listen!
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Citations below:
About BSE BSE (Bovine Spongiform Encephalopathy). Centers for Disease Control and Prevention. Published 2019. https://www.cdc.gov/prions/bse/about.html

Legname G. Elucidating the function of the prion protein. True HL, ed. PLOS Pathogens. 2017;13(8):e1006458. doi:10.1371/journal.ppat.1006458

Jung MJ, Pistolesi D, Panà A. Prions, prion diseases and decontamination. Igiene E Sanita Pubblica. 2003;59(5):331-344. https://pubmed.ncbi.nlm.nih.gov/14981553/

Lanska DJ. The mad cow problem in the UK: risk perceptions, risk management, and health policy development. Journal of Public Health Policy. 1998;19(2):160-183. Accessed April 11, 2022. https://pubmed.ncbi.nlm.nih.gov/9670700/

MRI used to detect vCJD. CMAJ: Canadian Medical Association Journal. 2000;163(3):324. Accessed April 11, 2022. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC80326/

Macfarlane RG, Wroe SJ, Collinge J, Yousry TA, Jäger HR. Neuroimaging findings in human prion disease. Journal of Neurology, Neurosurgery, and Psychiatry. 2007;78(7):664-670. doi:10.1136/jnnp.2006.094821

NHS Choices. Overview - Creutzfeldt-Jakob disease. NHS. Published 2019. https://www.nhs.uk/conditions/creutzfeldt-jakob-disease-cjd/

‌Mead S, Khalili-Shirazi A, Potter C, et al. Prion protein monoclonal antibody (PRN100) therapy for Creutzfeldt–Jakob disease: evaluation of a first-in-human treatment programme. The Lancet Neurology. 2022;21(4):342-354. doi:10.1016/s1474-4422(22)00082-5

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