A prion (IPA: /ˈpriːɒn/listen (help·info)) — combination of the first two syllables of the wors proteinaceous and infectious (-on by analogy to virion) — is a poorly-understood hypothetical infectious agent that, according to the "protein only" hypothesis, is composed entirely of proteins. Prions are thought to cause a number of diseases in a variety of mammals, including bovine spongiform encephalopathy (BSE, also known as "mad cow disease") in cattle and Creutzfeldt-Jakob disease (CJD) in humans. All thus-far hypothesized prion diseases affect the structure of the brain or other neural tissue, and all are currently untreatable and thought to be fatal. In general usage, prion can refer to both the theoretical unit of infection or the specific protein (e.g. PrP) that is thought to be the infective agent, whether or not it is in an infective state.