What is a prion?
Susan Lindquist is a researcher at the Howard Hughes Medical Institute, located in the department of molecular genetics and cell biology at the University of Chicago. She responds: “‘Prion’ is a term first used to describe the mysterious infectious agent responsible for several neurodegenerative diseases found in mammals, including Creutzfeldt-Jakob disease (CJD) in humans. The word itself derives from ‘proteinaceous infectious particle’; it refers to the initially heretical hypothesis that the infectious agent causing those diseases consists only of protein, with no nucleic acid genome. (All previously known pathogens, such as bacteria and viruses, contain nucleic acids, which enable them to reproduce.) The prion hypothesis explained why the mysterious infectious agent is resistant to ultraviolet radiation, which breaks down nucleic acids, but is susceptible to substances that disrupt proteins. “A major breakthrough occurred when researchers discovered that the infectious agent consis
Specifically, what is known about the molecular structure of prions and how they cause infections such as Creutzfeldt-Jakob disease? Susan Lindquist is a researcher at the Howard Hughes Medical Institute, located in the department of molecular genetics and cell biology at the University of Chicago. She responds: “‘Prion’ is a term first used to describe the mysterious infectious agent responsible for several neurodegenerative diseases found in mammals, including Creutzfeldt-Jakob disease (CJD) in humans. The word itself derives from ‘proteinaceous infectious particle’; it refers to the initially heretical hypothesis that the infectious agent causing those diseases consists only of protein, with no nucleic acid genome. (All previously known pathogens, such as bacteria and viruses, contain nucleic acids, which enable them to reproduce.) The prion hypothesis explained why the mysterious infectious agent is resistant to ultraviolet radiation, which breaks down nucleic acids, but is suscept
A prion is a nonliving, self-replicating infectious agent made of protein. It can reproduce with the aid of its host’s biological machinery, like a virus. “Prion” is short for “proteinaceous infectious particle.” The idea of an infectious agent lacking nucleic acids for reproduction was initially met with skepticism when the idea was proposed in the 60s, because such a phenomenon had never been observed before. While self-replicating nucleic acid-based entities are well-understood, the mechanism of self-replication among prions is still a mystery. Prions found in animals exclusively infect the brain, are fatal and untreatable. Prions have been discovered in fungi, but rather than damaging them, it is theorized that they may play a symbiotic role in the fungi’s immune system. Prions are responsible for the outbreak of Mad Cow Disease in Britain during the early 00s, which led to the destruction of thousands of livestock, and international fear about the possibility of the disease spread
A prion is a disease-causing agent, discovered by Stanley Prusiner, which is responsible for various fatal neurodegenerative diseases called transmissible spongiform encephalopathies. Prions are an abnormal form of a normally harmless protein found in mammals and birds. These abnormal proteins can enter the brain through infection, usually after being ingested, or it can arise from a mutation in the gene that encodes the protein. Unlike all other types of infectious agents in that they appear to lack nucleic acid (DNA or RNA). Once present in the brain, prions cause normal proteins to refold into abnormal shapes. As these abnormal proteins multiply, they destroy neurons and eventually cause brain tissue to become riddled with holes. Prions can only be destroyed through incineration.
