What is prion disease? Explain not for a biologist in clear language

I'll try to explain the mechanism of prion diseases on my fists. In the case of external infection, a prion protein enters the human body.Let's take a prion protein called PrPSc as an example. It enters the body, then in some way manages to get into the neuron and there it begins to modify the normal protein (let's call it PrPc), turning it into a similar one - PrPSc.

That is, once again, quite primitive: there is a "lopsided" protein -prion, which enters the nerve cell, there begins to convert the normal proteins of the nerve cell into lopsided ones. The cell cannot do anything with this lumpy protein - it cannot destroy, it cannot remove it. This protein accumulates in excessive amounts and leads to dysfunction of the cell and then to its death. This process is unhurried, but irreversible.

There is another - non-infectious way of development of prion diseases - genetic. In this case, there is a mutation of a certain gene, which leads to the fact that a protein with prion properties is synthesized. This protein also converts other proteins into prions. As a result, cell dystrophy and its death.

Prions are proteins that supposedly have a disease-causing effect.

Such diseases develop extremely slowly, and the method of reproduction of the pathogen and its infection has not been established. The only widely known "prion disease" is spongiform encephalopathy, or mad cow disease. Its existence also raises many questions - it arose and disappeared as soon as the competitive environment in European animal husbandry disappeared.

Therefore, in most cases, the very existence of pathogenic "prions" is doubtful.