Exploring myelin dysfunction in multiple system atrophy.

摘要: Multiple system atrophy (MSA) is a rare, yet fatal neurodegenerative disease that presents clinically with autonomic failure in combination parkinsonism or cerebellar ataxia. MSA impacts on the nervous affecting blood pressure, heart rate and bladder function, motor balance muscle movement. The cause of unknown, no definitive risk factors have been identified, there cure effective treatment. pathology presence α-synuclein aggregates brain therefore classified as an α-synucleinopathy, together Parkinson's dementia Lewy bodies. Although molecular mechanisms misfolding, fibrillation aggregation partly overlap other α-synucleinopathies, pathological pathway unique principal site for deposition oligodendrocytes rather than neurons. sequence events now recognized abnormal protein redistributions first, followed by myelin dysfunction then neurodegeneration. Oligodendrocytes are responsible production maintenance myelin, specialized lipid membrane encases axons all neurons brain. Myelin composed lipids two prominent proteins, basic proteolipid protein. In vitro studies suggest aberration distribution transport may lead to MSA. purpose this perspective bring available evidence explore potential role α-synuclein, dysfunction, dyshomeostasis ABCA8 pathogenesis.