Abstract：
The light-sensitive outer segment organelle of photoreceptor cells contains a stack of hundreds of flat, disc-shaped membranes called discs. The rims of these discs contain a photoreceptor-specific tetraspanin protein peripherin-2 (also known as rds or PRPH2). Mutations in the PRPH2 gene lead to a wide variety of inherited retinal degenerations in humans. The vast majority of these mutations occur within a large, intradiscal loop of peripherin-2, known as the D2 loop. The D2 loop mediates well-established intermolecular interactions of peripherin-2 molecules among themselves and a homologous protein ROM1. These interactions lead to the formation of large, highly ordered oligomers. In this chapter, we discuss the supramolecular organization of peripherin-2/ROM1 complexes and their contribution to the process of outer segment disc morphogenesis and enclosure.
摘要：
感光细胞的光敏外部细胞器包含数百个扁平的堆叠，圆盘形膜称为圆盘。这些圆盘的边缘含有光感受器特异性四跨膜蛋白paterin-2（也称为rds或PRPH2）。PRPH2基因中的突变导致人类中各种各样的遗传性视网膜变性。这些突变绝大多数发生在一个大的，周围蛋白-2的椎间盘内环，称为D2环。D2环介导外周蛋白2分子之间和同源蛋白ROM1之间的公认的分子间相互作用。这些相互作用导致大的形成，高度有序的低聚物。在这一章中,我们讨论了外周蛋白2/ROM1复合物的超分子组织及其在外段椎间盘形态发生和封闭过程中的贡献。
