Carrella Sabrina, Massa Filomena, Indrieri Alessia
Telethon Institute of Genetics and Medicine, Naples, Italy.
Institute for Genetic and Biomedical Research, National Research Council (CNR), Milan, Italy.
Front Cell Dev Biol. 2021 Jun 18;9:653522. doi: 10.3389/fcell.2021.653522. eCollection 2021.
The retina is among the most metabolically active tissues with high-energy demands. The peculiar distribution of mitochondria in cells of retinal layers is necessary to assure the appropriate energy supply for the transmission of the light signal. Photoreceptor cells (PRs), retinal pigment epithelium (RPE), and retinal ganglion cells (RGCs) present a great concentration of mitochondria, which makes them particularly sensitive to mitochondrial dysfunction. To date, visual loss has been extensively correlated to defective mitochondrial functions. Many mitochondrial diseases (MDs) show indeed neuro-ophthalmic manifestations, including retinal and optic nerve phenotypes. Moreover, abnormal mitochondrial functions are frequently found in the most common retinal pathologies, i.e., glaucoma, age-related macular degeneration (AMD), and diabetic retinopathy (DR), that share clinical similarities with the hereditary primary MDs. MicroRNAs (miRNAs) are established as key regulators of several developmental, physiological, and pathological processes. Dysregulated miRNA expression profiles in retinal degeneration models and in patients underline the potentiality of miRNA modulation as a possible gene/mutation-independent strategy in retinal diseases and highlight their promising role as disease predictive or prognostic biomarkers. In this review, we will summarize the current knowledge about the participation of miRNAs in both rare and common mitochondria-mediated eye diseases. Definitely, given the involvement of miRNAs in retina pathologies and therapy as well as their use as molecular biomarkers, they represent a determining target for clinical applications.
视网膜是代谢最活跃、能量需求高的组织之一。视网膜各层细胞中线粒体的特殊分布对于确保光信号传递所需的适当能量供应至关重要。光感受器细胞(PRs)、视网膜色素上皮(RPE)和视网膜神经节细胞(RGCs)中线粒体高度集中,这使得它们对线粒体功能障碍特别敏感。迄今为止,视力丧失与线粒体功能缺陷密切相关。许多线粒体疾病(MDs)确实表现出神经眼科症状,包括视网膜和视神经表型。此外,在最常见的视网膜病变,即青光眼、年龄相关性黄斑变性(AMD)和糖尿病视网膜病变(DR)中,经常发现线粒体功能异常,这些病变与遗传性原发性MDs有临床相似之处。微小RNA(miRNAs)是多种发育、生理和病理过程的关键调节因子。视网膜变性模型和患者中miRNA表达谱失调,突显了miRNA调节作为视网膜疾病中一种可能的不依赖基因/突变的策略的潜力,并突出了它们作为疾病预测或预后生物标志物的前景作用。在本综述中,我们将总结目前关于miRNAs在罕见和常见的线粒体介导的眼部疾病中的参与情况的知识。毫无疑问,鉴于miRNAs参与视网膜病变和治疗以及它们作为分子生物标志物的用途,它们是临床应用的决定性靶点。