Lipid dysfunction and pathogenesis of multiple system atrophy.

摘要: Multiple system atrophy (MSA) is a progressive neurodegenerative disease characterized by the accumulation of α-synuclein protein in cytoplasm oligodendrocytes, myelin-producing support cells central nervous (CNS). The brain most lipid-rich organ body and disordered metabolism various lipid constituents increasingly recognized as an important factor pathogenesis several diseases. α-Synuclein 17 kDa with close association to membranes biosynthetic processes CNS, yet its precise function matter speculation, particularly oligodendrocytes. aggregation neurons well-characterized feature Parkinson’s dementia Lewy bodies. Epidemiological evidence vitro studies molecular dynamics suggest that homeostasis may play role aggregation. However, MSA distinct from other α-synucleinopathies number respects, not least disparate cellular focus pathology. recent identification causal mutations polymorphisms COQ2, gene encoding enzyme for production lipid-soluble electron carrier coenzyme Q10 (ubiquinone), puts membrane transporters pathogenesis, although how such are involved early myelin degeneration observed remains unclear. purpose this review bring together available explore potential dyshomeostasis MSA. We hypothesize dysregulation specialized synthesis maintenance oligodendrocytes underlies unique neuropathology