Prion (proteinaceous infectious particle)

Abnormal or  malformed (or misfolded) version of normal cellular proteins that is believed to play a role in various neurological disorders in humans (e.g., Creutzfeldt-Jacob disease) and in animals such as sheep (scrapie) and cattle (bovine spongiform encephalopathy).  A hundred times smaller than a virus, it is neither a virus nor a bacterium and does not contain nucleic acid, yet nevertheless can self-replicate.  In humans, the disease can be sporadic, inherited or acquired.  Most cases are sporadic in nature in that they arise spontaneously without any known cause.  For sporadic CJD, the incidence about 1 in 1 million per year.  It can be localised to specific populations (e.g., kuru) .  The disease can in very rare cases have an onset during childhood via familial inheritance.  There is no know treatment.  The term ‘prion’ was coined by Stanley B. Pruisiner in 1982. .  

See Alzheimer’s disease, Nucleic acid, Protein folding problem, Proteins