alpha -Synuclein has risen to prominence during the past 5 years because of
its association with several neurodegenerative diseases that have come to
be known as the synucleinopathies: The clinical phenotype of the synucleino
pathies is variable, with the most common being parkinsonism, autonomic dys
function, and dementia. Progress has been made in clinical, neuropathologic
and biochemical characterization of the synucleinopathies and their differ
entiation from other neurodegenerative disorders. At the molecular level, t
he synucleinopathies have conformational and post-translational modificatio
ns of synuclein that favor its fibrillization and aggregation in inclusions
in neurons and glia. Whether inclusion body formation is an adaptive respo
nse or is directly related to degeneration of neuronal and glial cells is a
topic of current research. Curr Opin Neurol 14:423-432. (C) 2001 Lippincot
t Williams & Wilkins.