Molecular genetic analyses have elucidated a class of inherited neurod
egenerative disorders caused by expanded CAG repeats encoding polyglut
amines (e.g. Huntington disease and Machado Joseph disease). Proteins
containing expanded polyglutamine repeats appear to precipitate by sel
f-aggregation and as a result, produce a core disease-related phenotyp
e: neuronal cell death or degeneration. In other neurodegenerative dis
orders, such as Alzheimer disease, prion disease, Parkinson disease an
d amyotrophic lateral sclerosis, precipitation of abnormal proteins is
also now considered to play a key role. These observations might lead
to the elucidation of universal mechanisms for neurodegeneration and
to effective treatments for many neurordegenerative disorders.