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Hacettepe Üniversitesi, Biyoloji Bölümü, Moleküler Biyoloji A.D., Ankara Retinitis pigmentosa (RP) is a heterogeneous group of retinal dystrophies that is characterized by photoreceptor degeneration. RP causes night blindness, a gradual loss of peripheral visual fields and eventual loss of central vision. The disease can be inherited in autosomal dominant, autosomal recessive, Xlinked and digenic modes. X-linked retinitis pigmentosa (XLRP) is one of the severe forms of progressive retinal degeneration. Five loci have been mapped for XLRP ; RP2, RP3, RP6, RP23 and RP24. Among these, RP3 accounts for 70%-90% and RP2 accounts for 10%-20% of genetically identifiable disease. Mutations in the RPGR gene are associated with RP3 subtype of XLRP. Mutations in RPGR have been identified in exons 1-14; however, ORF15 is the hot spot for mutations accounting for 50-60% of XLRP in Europe and North America. ORF15 mutations have also been detected in simplex RP males. Here we describe XLRP and the molecular analysis of RPGR and RP2 genes responsible for the disease condition. Keywords : Retinitis pigmentosa, XLRP, RPGR, RP2, retina, molecular analysis, retinal degenerations