Yusuf IH, Birtel J, Shanks ME, et al. - In this retrospective, case-series study conducted at two tertiary referral centers for inherited retinal diseases of nine consecutive individuals from eight families with retinitis pigmentosa (RP) attributed to variants in SNRNP200 (recently identified genetic cause of autosomal dominant RP), experts explained the retinal phenotype in individuals with RP secondary to variants in SNRNP200. Data imply that variants in SNRNP200 lead to nonsyndromic RP with a typical phenotype of rod-predominant dystrophy. Within some afflicted families, important phenotypic heterogeneity and nonpenetrance were observed. Moreover, with slow progression and well-preserved visual acuities into the fifth and sixth decades, symptom onset was notably within the first two decades of life.