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Review: Multiple system atrophy: emerging targets for interventional therapies.

ABSTRACT: Multiple system atrophy (MSA) is a fatal orphan neurodegenerative disorder that manifests with rapidly progressive autonomic and motor dysfunction. The disease is characterized by the accumulation of ?-synuclein fibrils in oligodendrocytes that form glial cytoplasmic inclusions, a neuropathological hallmark and central player in the pathogenesis of MSA. Here, we summarize the current knowledge on the etiopathogenesis and neuropathology of MSA. We discuss the role of ?-synuclein pathology, microglial activation, oligodendroglial dysfunction and putative cell death mechanisms as candidate therapeutic targets in MSA.

SUBMITTER: Stefanova N 

PROVIDER: S-EPMC4788141 | biostudies-other | 2016 Feb

REPOSITORIES: biostudies-other

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Review: Multiple system atrophy: emerging targets for interventional therapies.

Stefanova N N   Wenning G K GK  

Neuropathology and applied neurobiology 20160201 1

Multiple system atrophy (MSA) is a fatal orphan neurodegenerative disorder that manifests with rapidly progressive autonomic and motor dysfunction. The disease is characterized by the accumulation of α-synuclein fibrils in oligodendrocytes that form glial cytoplasmic inclusions, a neuropathological hallmark and central player in the pathogenesis of MSA. Here, we summarize the current knowledge on the etiopathogenesis and neuropathology of MSA. We discuss the role of α-synuclein pathology, microg  ...[more]

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