You’ve probably heard of prion diseases before, even if you don’t recognize the term ‘prion’ or know the pathology behind them. Mad cow disease stirred the media into a frenzy in the past few decades, and Kuru is often mentioned in conjuncture with cannibalism. The cause of these diseases, prions are one of the most bizarre and fascinating pathogens around. They’re nigh indestructible and even prion ash is infectious. They’re pathogens that are nonliving, a trait which contradicts most biological disease lore. In a further twist, a single prion disease can be infectious, sporadic, and genetic—most diseases only have a single cause. So, what exactly is a prion and how does it cause these diseases? Surprisingly, it all comes down to a single, misfolded protein.
What is a prion?
The human body has around a hundred thousand types of proteins, which are often described as the building blocks of organisms. DNA, the genetic material in all cells, is used to make RNA, which is then translated into proteins: ribbons of amino acids. These ribbons fold according to various biochemical and physical laws into distinct and specific shapes that allow them to carry out their varied purposes, from structural to catalyzing chemical reactions [6]. A mutation in a gene, a segment of DNA encoding a protein, can therefore completely change or disrupt the function of a protein and have disastrous consequences.
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