Transcription
This is a pron, one of the most feared molecules on the planet. It doesn't just infect you, it rewrites you to make infinite copies of itself. And in one family, it rewrote something essential out of existence. The ability to sleep, not for a night, but forever. This is the story of the family that forgot how to sleep. Welcome back to Scientifically Interesting Ways to Die.
While on a cruise ship sailing the Mediterranean Sea, a 53-year-old man named Sano was embarrassed to find that his shirt had suddenly become drenched in sweat. Looking in the mirror, his pupils had shrunk to tiny pimprics, the same glossy stare he'd seen in his father and his sister before him, both of whom had died in middle age.
Returning home, Sano couldn't sleep. Not just poor sleep, but literally no sleep at all. He would begin to drift off, only to jolt awake in panic. His body had started to break down. SNO took himself to the University of Bolognia's sleep clinic seeking answers. The external symptoms were terrifying, but what was happening inside was a nightmare.
Passed down through his family located on chromosome 20 was a single point mutation D178N in the PRNP gene, which encoded for PON protein PRP. But this single mutation causes the protein to misfold into a toxic shape. Even worse, it triggers other pron proteins to misfold too, starting a chain reaction through the brain that kills neurons in their wake, specifically those in the phalamus that regulate sleep wake cycles, autonomic functions, and emotional processing.
As the weeks passed, Sana began to hallucinate. He became disoriented and paranoid, and he'd act out familiar rituals in semic-conscious states, pretending to comb his hair or button an imaginary shirt. The elegant man who cruised the Mediterranean now stumbled through the hospital's halls in a gown, confused and afraid. By July of 1984, Sana was emaciated, unable to eat and speak, and within a few weeks, he fell into a state of ainetic mutism, awake, but unresponsive. Not long after, he died from exhaustion.
Sano donated his brain to science, and when doctors examined it, the damage was unmistakable. The phalamus was riddled with holes as if eaten from the inside out. When neurons had been destroyed, it left behind a sponge-like landscape where misfolded prons had silently done their work. But the most terrifying part, prons have been shown to survive traditional sterilization approaches from autoclaving to bleach. And they're capable of passing from one patient to another on contaminated surgical instruments. Neither alive nor dead, the prons that killed Silvano are still active in his preserved brain tissue even decades later.
Thanks to his case, researchers at the University of Bologna were able to identify and name this new and deadly condition, fatal familial insomnia. Only about 70 families in the world carry this fateful mutation. And today, there still is no cure. So, if you find yourself tossing and turning at 3:00 a.m. worrying about your nighttime routine, just remember, a good night's sleep starts with no caffeine, low blue light, and a functioning phalamus. If you like science that keeps you up at night, follow for