Alpha-synuclein and the Lewy body disorders.
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 synucleinopathies is variable, with the most common being parkinsonism, autonomic dysfunction, and dementia. Progress has been made in clinical, neuropathologic and biochemical characterization of the synucleinopathies and their differentiation from other neurodegenerative disorders. At the molecular level, the synucleinopathies have conformational and post-translational modifications of synuclein that favor its fibrillization and aggregation in inclusions in neurons and glia. Whether inclusion body formation is an adaptive response or is directly related to degeneration of neuronal and glial cells is a topic of current research.
Dickson
D W
DW
Department of Pathology, Mayo Clinic, Jacksonville, Florida 32224, USA. dickson.dennis@mayo.edu
D003937:Diagnosis, Differential; D006801:Humans; D002479:Inclusion Bodies / Q000473:pathology; D020961:Lewy Body Disease / Q000235:genetics* / Q000473:pathology; D009419:Nerve Tissue Proteins / Q000235:genetics*; D009457:Neuroglia / Q000473:pathology; D009474:Neurons / Q000473:pathology; D010641:Phenotype; D051843:Synucleins; D051844:alpha-Synuclein
D009419:Nerve Tissue Proteins; C497604:SNCA protein, human; D051843:Synucleins; D051844:alpha-Synuclein