Fifteen years ago I evoked a good deal of skepticism when I proposed that the infectious agents causing certain degenerative disorders of the central nervous system in animals and, more rarely, in humans might consist of protein and nothing else. At the time, the notion was heretical. Dogma held that the conveyers of transmissible diseases required genetic material, composed of nucleic acid (DNA or RNA), in order to establish an infection in a host. Even viruses, among the simplest microbes, rely on such material to direct synthesis of the proteins needed for survival and replication.
Later, many scientists were similarly dubious when my colleagues and I suggested that these "proteinaceous infectious particles"--or "prions," as I called the disease-causing agents--could underlie inherited, as well as communicable, diseases. Such dual behavior was then unknown to medical science. And we met resistance again when we concluded that prions (pronounced "pree-ons") multiply in an incredible way; they convert normal protein molecules into dangerous ones simply by inducing the benign molecules to change their shape.
This article was originally published with the title The Prion Diseases.