PRPH2 (Peripherin-2) variants and mutations

PRPH2 (also known as Peripherin-2) is a human protein-coding gene encoding a peripherin-2 protein. It organizes and stabilizes the rim structure of photoreceptor outer-segment discs. Pathogenic variants cause a wide range of inherited retinal diseases including retinitis pigmentosa, pattern dystrophy, and macular dystrophy. This analysis covers 910 PRPH2 variants and mutations. Of these, 86% have computational variant effect predictions. Disease context includes retinitis pigmentosa, patterned macular dystrophy 1, and vitelliform macular dystrophy 3. Example PRPH2 variants include M1L, M1R, and M1T.

Variant analysis overview

Variant and mutation evidence

Clinical, disease, and population context

Protein structure and variant hotspots

Data sources

Evidence in this analysis draws on EBI Proteins Variation, UniProt, gnomAD v4, EuropePMC, 3D Hotspot Analysis, Interaction Network Analysis, Protein Data Bank, AlphaFold DB, gnomAD constraint, Open Targets, MaveDB, LitVar.

Notable PRPH2 variants

Examples include M1L, M1R, M1T, M1V, A2E, A2S, A2T, A2V. Listed records include available protein-change notation, database identifiers, clinical classifications, computational predictions, population evidence, experimental measurements, and disease context.