Alpha-synuclein: normal function and role in neurodegenerative diseases.
Synucleins are a family of small, highly charged proteins expressed predominantly in neurons. Since their discovery and characterization during the last decade, much has been learned about their structure, potential functions, interactions with other proteins, and roles in disease. One of these proteins, alpha-synuclein (alpha-syn), is the major building block of pathological inclusions that characterize many neurodegenerative disorders, including Parkinson's disease (PD), dementia with Lewy bodies (DLB), and neurodegeneration with brain iron accumulation type 1 (NBIA-1), which collectively are termed synucleinopathies. Furthermore, genetic and biological studies support a role for alpha-syn in the pathophysiology of these diseases. Therefore, research must be continued in order to better understand the functions of the synuclein proteins under normal physiological conditions as well as their role in diseases.
Norris
Erin H
EH
Center for Neurodegenerative Disease Research and the Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104, USA.
Giasson
Benoit I
BI
Lee
Virginia M-Y
VM
D000595:Amino Acid Sequence; D000818:Animals; D030801:Animals, Genetically Modified; D003704:Dementia / Q000378:metabolism; D006801:Humans; D016631:Lewy Bodies / Q000473:pathology; D008954:Models, Biological; D008956:Models, Chemical; D008969:Molecular Sequence Data; D019578:Multiple System Atrophy / Q000378:metabolism; D009419:Nerve Tissue Proteins / Q000378:metabolism / Q000502:physiology*; D019636:Neurodegenerative Diseases / Q000378:metabolism*; D009584:Nitrogen / Q000378:metabolism; D010100:Oxygen / Q000378:metabolism; D010300:Parkinson Disease / Q000378:metabolism; D010575:Pesticides / Q000009:adverse effects; D011485:Protein Binding; D017386:Sequence Homology, Amino Acid; D051843:Synucleins; D051844:alpha-Synuclein
D009419:Nerve Tissue Proteins; D010575:Pesticides; C497604:SNCA protein, human; D051843:Synucleins; D051844:alpha-Synuclein; D009584:Nitrogen; D010100:Oxygen