학술논문

Role of Oligodendrocyte Lineage Cells in Multiple System Atrophy.
Document Type
Article
Source
Cells (2073-4409). Mar2023, Vol. 12 Issue 5, p739. 18p.
Subject
*MULTIPLE system atrophy
*OLIGODENDROGLIA
*ALPHA-synuclein
*MOVEMENT disorders
*PROGENITOR cells
Language
ISSN
2073-4409
Abstract
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 α-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 α-synuclein, and discuss the postulated mechanisms of how oligodendrogliopathy develops, oligodendrocyte progenitor cells as the potential origins of the toxic seeds of α-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. [ABSTRACT FROM AUTHOR]