Abstract：
The outer segments of photoreceptors are specialized sensory cilia crucial for light detection. Any disruption that alters outer segment morphology can impair photoreceptor function and therefore vision. Progressive rod-cone degeneration (PRCD) is an integral membrane protein exclusively present in the photoreceptor OS with an unknown function. Multiple mutations in PRCD are linked with retinitis pigmentosa. The most common PRCD mutation observed in both human and multiple dog breeds, PRCD-C2Y, lacks the lipid modification \"palmitoylation,\" which is crucial for protein stability and trafficking to the OS. Previous studies including ours show impaired disc morphogenesis and rhodopsin distributions in the absence of PRCD, but the precise role of PRCD in maintaining OS structure and function remains unclear. In this chapter, we discuss the potential role of PRCD in the maintenance of photoreceptor OS structural and functional integrity.
摘要：
光感受器的外部部分是对光检测至关重要的特殊感觉纤毛。任何改变外节形态的破坏都会损害光感受器功能，从而损害视力。进行性视锥细胞变性（PRCD）是一种完整的膜蛋白，仅存在于光感受器OS中，具有未知的功能。PRCD中的多个突变与色素性视网膜炎有关。在人类和多个犬种中观察到的最常见的PRCD突变，PRCD-C2Y,缺乏脂质修饰“棕榈酰化，“这对于蛋白质的稳定性和向OS的运输至关重要。包括我们在内的先前研究表明，在没有PRCD的情况下，椎间盘形态发生和视紫红质分布受损，但是PRCD在维护操作系统结构和功能方面的确切作用尚不清楚。在这一章中,我们讨论了PRCD在维持感光器OS结构和功能完整性方面的潜在作用。
