POV: Prions Take Away Your Sleep

[00:00:00]This is a prion, a misfolded protein that can damage our cells. And the thing about prions that's unique in biology is that all known prion diseases, once they develop symptoms, are 100% fatal. But in a 53-year-old man named Silvano and his family, the way they did this was particularly brutal. So, here's his story. See, in 1983, the 53-year-old man was on a Mediterranean cruise ship when his body began being unable to let itself sleep. He'd be drifting off, and as soon as he was about to enter sleep, his body would jolt itself awake. The insomnia continued indefinitely, so he checked himself into the Bologna Sleep Clinic to see what was going on, not realizing that inside his body was a point mutation in chromosome 20, which led to the misfolded protein and a condition called fatal familial insomnia. After 46 months of progressive inability to sleep, his body began breaking down, and he eventually joined his parents and passed away. But he donated his brain to science, and the thalamus, which is an important part in regulating sleep-wake cycles, had actually been turned by the prion disease from a solid structure into a sponge-like one. His donation helped us to understand prion diseases more deeply, but there still is no cure. Had you heard this story before? Let us know.