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. 2023:1415:277-281.
doi: 10.1007/978-3-031-27681-1_40.

The Role of Peripherin-2/ROM1 Complexes in Photoreceptor Outer Segment Disc Morphogenesis

Tylor R Lewis  1 , Muayyad R Al-Ubaidi  2   3 , Muna I Naash  2   3 , Vadim Y Arshavsky  4   5
Affiliations

The Role of Peripherin-2/ROM1 Complexes in Photoreceptor Outer Segment Disc Morphogenesis

Tylor R Lewis et al. Adv Exp Med Biol. 2023.

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.

Keywords: Disc; Disc Enclosure; Disc Rim; Outer Segment; PRPH2; Peripherin-2; Photoreceptor; RDS; ROM1; Retina.

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