This mutation results in deregulation of intracellular calcium homeostasis that leads to photoreceptor cell death via apoptosis.27, –29 Mutations in the PDE6β gene also cause RP in humans accounting for approximately 5% of all autosomal dominant RP cases worldwide.30,31 Therefore, rd10 mice provide a useful and widely used model of RP that closely resembles the human disease emulating the progression of typical autosomal recessive RP. Here, PDE6B is linked to retinitis pigmentosa 1.