Episode 64. Metamorphosis – CJD and Other Brain Prion Shapeshifters

The focus of this podcast is the enigmatic theme of prion disorders. I trace the history of our understanding of this neurodegenerative disorder with how Nobel laureate D. Carleton Gajdusek, described the transmission of kuru - the first pathologically defined human prion disease.

I also narrate how the similarities in the clinical and pathological features of kuru and scrapie, the disease of sheep, opened the way to this breakthrough. I also chronicled how the efforts of another Nobel laureate, Stanley Prusiner, characterised the pathology, genetics and transmission of scrapie, and how he succeeded in a bitter scientific race that transformed the field of transmissible spongiform encephalopathies which he named prion diseases.

I also explored the pathology of prions, their normal functions, and how they cause disease when they beco ...