What exactly are prion diseases and how do people get them?

Question

What exactly are prion diseases and how do people get them?

In: 1

Answer 1

Prions are proteins that have gotten misfolded…we don’t know how, or why, but we do know that one misfolded protein can influence another to misfold, then another etc, etc…. This is how prion diseases spread.

Proteins start out as something called amino acids, which are strung together in a chain, like a beaded necklace. And similar to if you twist a beaded necklace around in your hand, it starts to coil up on itself, these amino acid chains fold in predictable ways to form proteins, which perform various functions. If a protein isn’t folded in the correct way, it won’t be able to interact with the other proteins that it’s supposed to, halting many of the bodies essential processes.

Prion diseases seem to arise due to cannibalism. It’s been observed across multiple mammalian species, including cows (Creutzfeld-Jakob aka Mad Cow), sheep (scrapies) and humans (Kuru). Notably with CJD, when cow feed has bovine bone meal in it, the cow can develop CJD, which then can pass to humans who consume the infected meat.

Answer 2

Prions are proteins that have gotten misfolded…we don’t know how, or why, but we do know that one misfolded protein can influence another to misfold, then another etc, etc…. This is how prion diseases spread.

Proteins start out as something called amino acids, which are strung together in a chain, like a beaded necklace. And similar to if you twist a beaded necklace around in your hand, it starts to coil up on itself, these amino acid chains fold in predictable ways to form proteins, which perform various functions. If a protein isn’t folded in the correct way, it won’t be able to interact with the other proteins that it’s supposed to, halting many of the bodies essential processes.

Prion diseases seem to arise due to cannibalism. It’s been observed across multiple mammalian species, including cows (Creutzfeld-Jakob aka Mad Cow), sheep (scrapies) and humans (Kuru). Notably with CJD, when cow feed has bovine bone meal in it, the cow can develop CJD, which then can pass to humans who consume the infected meat.