The Mechanism of Endoplasmic Reticulum Proteostasis and Proteotoxicity in Retinal Degeneration

内质网膜蛋白复合物（EMC）是内质网稳态维持和膜蛋白生物生成的重要因子。使用一个缺乏emc的斑马鱼模型，我发现突变的鱼表现出各种视网膜表型，包括光感受器细胞的丧失和视觉功能的缺乏。此外，在携带实时监测UPR激活的报告基因的emc缺陷斑马鱼中，我发现UPR早在受精后3天（dpf）就被激活。这一发现进一步被大量RNA测序分析和qPCR分析证实。这些数据表明内质网应激和UPR可能在调节视网膜变性相关的emc功能障碍中发挥关键作用。

ER membrane protein complex (EMC) is an important factor for ER homeostasis maintenance and membrane protein biogenesis. Using an EMC-deficient zebrafish model, I found that mutant fish display various retinal phenotypes, including the loss of photoreceptor cells and lack of visual function. Additionally, an EMC-deficient zebrafish carrying a reporter gene that monitor UPR activation in real time, I found that UPR is activated as early as 3 days post fertilization (dpf). This finding is further confirmed with bulk RNA sequencing analysis and qPCR analysis. These data suggest that ER stress and UPR may play a critical role in regulating retinal degeneration associated with EMC-dysfunction.