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Multiple System Atrophy : Pathology, Pathogenesis, and Path Forward
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DOI：10.1146/annurev-pathmechdis-051122-104528.png)
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Abstract
En 中文
Multiple system atrophy (MSA) is a fatal neurodegenerative disease characterized by autonomic failure and motor impairment. The hallmark pathologic finding in MSA is widespread oligodendroglial cytoplasmic inclusions rich in aggregated a-synuclein (aSyn). MSA is widely held to be an oligodendroglial synucleinopathy, and we outline lines of evidence to support this assertion, including the presence of early myelin loss. However, we also consider emerging data that support the possibility of neuronal or immune dysfunction as a primary driver of MSA. These hypotheses are placed in the context of a major recent discovery that aSyn is conformationally distinct in MSA versus other synucleinopathies such as Parkinson's disease. We outline emerging techniques in epidemiology, genetics, and molecular pathology that will shed more light on this mysterious disease. We anticipate a future in which cutting-edge developments in personalized disease modeling, including with pluripotent stem cells, bridge mechanistic developments at the bench and real benefits at the bedside.
Keywords:
multiple system atrophy
alpha-synuclein
alpha-synucleinopathy
neuroinflammation
oligodendrocyte
myelin
Journal
A
IF:
34.5
Papers:
392
Citations:
9.5K
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