高级蛋白质组装体(通常称为蛋白质聚集体)的形成长期以来与疾病状态有关,特别是神经退行性疾病。在眼睛内,蛋白质聚集也涉及各种视网膜变性疾病,从视网膜色素变性(RP)到MalattiaLeventinese/Doyne蜂窝视网膜营养不良(ML/DHRD)到年龄相关性黄斑变性(AMD)。然而,许多重要的细胞过程,包括转录,翻译,非膜结合细胞器的形成需要功能性的形成,非病理性蛋白质聚集体维持细胞稳态。因此,功能性蛋白质聚集体,也称为冷凝物,可能在维持正常的视网膜功能中发挥重要作用。然而,目前,在我们的知识中存在一个关键的差距:在正常条件下,哪些蛋白质在视网膜内形成高阶组装体,这些结构起什么作用?我们提供的数据表明,蛋白质聚集在正常的多个视网膜层中是可识别的,健康的鼠视网膜,并简要讨论聚集蛋白对正常视网膜功能的潜在贡献,重点放在感光体内段和外段。
The formation of higher-order protein assemblies (commonly called protein aggregates) has long been associated with disease states, particularly in neurodegenerative disorders. Within the eye, protein aggregation has also been implicated in various retinal degenerative diseases ranging from retinitis pigmentosa (RP) to Malattia Leventinese/Doyne Honeycomb Retinal Dystrophy (ML/DHRD) to age-related macular degeneration (AMD). Yet, many essential cellular processes including transcription, translation, and the formation of non-membrane bound organelles require the formation of functional, non-pathologic protein aggregates to maintain cellular homeostasis. Thus, functional protein aggregates, also called condensates, likely play essential roles in maintaining normal retina function. However, currently, there is a critical gap in our knowledge: What proteins form higher-order assemblies under normal conditions within the retina and what function do these structures serve? Herein, we present data suggesting that protein aggregation is identifiable in multiple retinal layers of normal, healthy murine retina, and briefly discuss the potential contributions of aggregated proteins to normal retinal function, with a focus on the photoreceptor inner and outer segment.