α-Synucleinopathy phenotypes.

Abstract

α-Synucleinopathies are neurodegenerative diseases characterised by the abnormal accumulation of α-synuclein aggregates in neurons, nerve fibres or glial cells. While small amounts of these α-synuclein pathologies can occur in some neurologically normal individuals who do not have associated neurodegeneration, the absence of neurodegeneration in such individuals precludes them from having a degenerative α-synucleinopathy, and it has yet to be established whether such individuals have a form of preclinical disease. There are three main types of α-synucleinopathy, Parkinson's disease (PD), dementia with Lewy bodies (DLB), and multiple system atrophy (MSA), with other rare disorders also having α-synuclein pathologies, such as various neuroaxonal dystrophies. Multiple clinical phenotypes exist for each of the three main α-synucleinopathies, with these phenotypes differing in the dynamic distribution of their underlying neuropathologies. Identifying the factors involved in causing different α-synuclein phenotypes may ultimately lead to more targeted therapeutics as well as more accurate clinical prognosis.

• Authors: Heather McCann, Claire H Stevens, Heidi Cartwright, Glenda M Halliday
• Journal: Parkinsonism & related disorders (Parkinsonism Relat Disord) Vol. 20 Suppl 1 Pg. S62-7 (Jan 2014) ISSN: 1873-5126 [Electronic] England
• PMID: 24262191 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't, Review)
• Copyright: Copyright © 2013 Elsevier Ltd. All rights reserved.

Chemical References

• alpha-Synuclein

Topics

• Humans
• Lewy Body Disease (metabolism, pathology)
• Multiple System Atrophy (metabolism, pathology)
• Parkinson Disease (metabolism, pathology)
• Phenotype
• alpha-Synuclein (metabolism)