Alpha Synuclein in a Model of Multiple System Atrophy

摘要

Multiple system atrophy (MSA) is a progressive neurological disorder characterized by parkinsonism, cerebellar dysfunction and autonomic impairment. The cardinal pathological feature of MSA is the glial cytoplasmic inclusion (OCI) in oligodendrocytes, and a major component of OCIs is a-synuclein. We identified an oligodendrocytic precursor cell line, CG-4, that endogenously produces a-synuclein. We characterized alpha synuclein-immunoreactive (alpha synuclein-IR) material in the CO-4 cells (with affection to higher molecular weight forms), and studied the effects of oxidative stress. We also produced CO- 4 cells that constitutively overexpress human alpha-synuclein and CO-4 cells that express human alpha-synuclein under control of the tetracycline-ON (inducible promoter) system, and we have characterized both of these.