Role of Oligodendrocyte Lineage Cells in Multiple System Atrophy

卷:12

Review

关键词: GLIAL CYTOPLASMIC INCLUSIONS;    ALPHA-SYNUCLEIN;    PROGENITOR CELLS;    STRIATONIGRAL DEGENERATION;    TRANSCRIPTOMIC ANALYSIS;    CEREBROSPINAL-FLUID;    PARKINSONS-DISEASE;    REGIONAL IDENTITY;    NATURAL-HISTORY;    PRECURSOR CELLS;   

DOI  :  10.3390/cells12050739

来源: SCIE

【 摘 要 】

Multiple system atrophy (MSA) is a debilitating movement disorder with unknown etiology. Patients present characteristic parkinsonism and/or cerebellar dysfunction in the clinical phase, resulting from progressive deterioration in the nigrostriatal and olivopontocerebellar regions. MSA patients have a prodromal phase subsequent to the insidious onset of neuropathology. Therefore, understanding the early pathological events is important in determining the pathogenesis, which will assist with developing disease-modifying therapy. Although the definite diagnosis of MSA relies on the positive post-mortem finding of oligodendroglial inclusions composed of alpha-synuclein, only recently has MSA been verified as an oligodendrogliopathy with secondary neuronal degeneration. We review up-to-date knowledge of human oligodendrocyte lineage cells and their association with alpha-synuclein, and discuss the postulated mechanisms of how oligodendrogliopathy develops, oligodendrocyte progenitor cells as the potential origins of the toxic seeds of alpha-synuclein, and the possible networks through which oligodendrogliopathy induces neuronal loss. Our insights will shed new light on the research directions for future MSA studies.