Department of Clinical Medicine, Faculty of Medicine, Health and Human Sciences, Macquarie University, Wallumattagal Campus, NSW 2109, Australia.
Save Sight Institute, University of Sydney, Sydney, NSW 2000, Australia.
Aging Dis. 2024 Oct 1;15(5):2301-2314. doi: 10.14336/AD.2024.0336-1.
Glaucoma is a neurodegenerative disease affecting millions worldwide, characterised by retinal ganglion cell (RGC) degeneration which leads to blindness in more advanced cases. Although the pathogenesis and underlying mechanisms of glaucoma are not fully understood, there are theories that hint at demyelination playing a role in the disease process. Demyelination, or the degeneration of the myelin sheath surrounding axons, has been found in previous studies using animal models of glaucoma and clinical assessments of glaucoma patients. However, this has not been fully realised or quantified in glaucoma patients. Utilising postmortem optic nerve samples from glaucoma and healthy subjects, various immunohistochemical and morphological assessments were performed to determine the extent, if any, of demyelination in glaucomatous optic nerves. Our findings revealed that alongside nerve shrinkage and degeneration of nerve tissue fascicles, there were significantly less myelin proteins, specifically myelin basic protein (MBP), in glaucoma optic nerves. Additionally, the loss of MBP was correlated with decreased oligodendrocyte (OLG) precursors and increasing glial activity. This further supports previous evidence that demyelination may be a secondary degenerative process associated with glaucoma disease progression. Not only do these results provide evidence for potential disease mechanisms, but this is also the first study to quantify optic nerve demyelination in glaucoma postmortem tissue.
青光眼是一种影响全球数百万人的神经退行性疾病,其特征是视网膜神经节细胞(RGC)退化,在更严重的情况下会导致失明。尽管青光眼的发病机制和潜在机制尚未完全了解,但有理论表明脱髓鞘在疾病过程中起作用。脱髓鞘,即轴突周围髓鞘的退化,在使用青光眼动物模型和青光眼患者临床评估的先前研究中已经发现。然而,在青光眼患者中尚未完全实现或量化。利用青光眼和健康受试者的死后视神经样本,进行了各种免疫组织化学和形态学评估,以确定在青光眼视神经中是否存在脱髓鞘。我们的研究结果表明,除了神经收缩和神经组织束的退化外,青光眼视神经中的髓鞘蛋白,特别是髓鞘碱性蛋白(MBP)明显减少。此外,MBP 的丢失与少突胶质细胞(OLG)前体减少和神经胶质活性增加有关。这进一步支持了先前的证据,即脱髓鞘可能是与青光眼疾病进展相关的继发性退行性过程。这些结果不仅为潜在的疾病机制提供了证据,而且也是首次在青光眼死后组织中定量视神经脱髓鞘的研究。