Intermediate Laboratory of Gene Therapy and Viral Vectors, Carlos Chagas Filho Biophysics Institute, Federal University of Rio de Janeiro, Rio de Janeiro 21941-902, Brazil.
Department of Neurology, University Medical Center Göttingen, 37077 Göttingen, Germany.
Int J Mol Sci. 2022 Apr 1;23(7):3935. doi: 10.3390/ijms23073935.
Glaucoma is a neurodegenerative disease that affects the retinal ganglion cells (RGCs) and leads to progressive vision loss. The first pathological signs can be seen at the optic nerve head (ONH), the structure where RGC axons leave the retina to compose the optic nerve. Besides damage of the axonal cytoskeleton, axonal transport deficits at the ONH have been described as an important feature of glaucoma. Axonal transport is essential for proper neuronal function, including transport of organelles, synaptic components, vesicles, and neurotrophic factors. Impairment of axonal transport has been related to several neurodegenerative conditions. Studies on axonal transport in glaucoma include analysis in different animal models and in humans, and indicate that its failure happens mainly in the ONH and early in disease progression, preceding axonal and somal degeneration. Thus, a better understanding of the role of axonal transport in glaucoma is not only pivotal to decipher disease mechanisms but could also enable early therapies that might prevent irreversible neuronal damage at an early time point. In this review we present the current evidence of axonal transport impairment in glaucomatous neurodegeneration and summarize the methods employed to evaluate transport in this disease.
青光眼是一种神经退行性疾病，影响视网膜神经节细胞（RGC），导致进行性视力丧失。最早的病理迹象可见于视神经头（ONH），即 RGC 轴突离开视网膜组成视神经的结构。除了轴突细胞骨架的损伤外，ONH 处的轴突运输缺陷也被描述为青光眼的一个重要特征。轴突运输对于适当的神经元功能至关重要，包括细胞器、突触成分、囊泡和神经营养因子的运输。轴突运输的损伤与几种神经退行性疾病有关。青光眼轴突运输的研究包括在不同的动物模型和人类中的分析，并表明其失败主要发生在 ONH 中，并且在疾病进展的早期发生，早于轴突和胞体的退化。因此，更好地了解轴突运输在青光眼发病机制中的作用不仅对破译疾病机制至关重要，而且还可能实现早期治疗，以便在早期阶段防止不可逆转的神经元损伤。在这篇综述中，我们介绍了青光眼神经退行性变中轴突运输受损的现有证据，并总结了用于评估该疾病中运输的方法。
