Abstract

Mounting evidence argues that prions feature in the pathogenesis of many, if not all, neurodegenerative diseases. Such disorders include Alzheimer’s, Parkinson’s, Lou Gehrig’s and Creutzfeldt-Jakob diseases as well as the fronto-temporal dementias. In each of these illnesses, aberrant forms of a particular protein accumulate as pathological deposits referred to as amyloid plaques, neurofibrillary tangles, Lewy bodies, as well as glial cytoplasmic and/or nuclear inclusions. The heritable forms of the neurodegenerative diseases are often caused by mutations in the genes encoding the mutant, prion proteins that accumulate in the CNS of patients with these fatal disorders. The late onset of the inherited neurodegenerative diseases seems likely to be explained by the protein quality control systems being less efficient in older neurons and thus, more permissive for prion accumulation. To date, there is not a single drug that halts or even slows one neurodegenerative disease.