Analysis of alpha-synuclein aggregation and associated pathologies in a cellular model of multiple system atrophy

Intracellular aggregation of alpha-synuclein is a neuropathological hallmark of a variety of neurodegenerative diseases, including Parkinson's disease and multiple system atrophy (MSA). The deposition of the normally neuronal alpha-synuclein protein within (oligodendro)glial cytoplasmic inclusions (GCIs) and its relationship with oligodendrocyte death in MSA have not been elucidated. We developed a cellular model that recapitulates features of the degenerative process that occurs in MSA. Ectopic human alpha-synuclein expression in primary oligodendrocytes was achieved by deriving cultures from transgenic mice, w…