PDE6B (phosphodiesterase 6B) is a rod-specific cGMP phosphodiesterase that catalyzes hydrolysis of 3',5'-cyclic GMP, essential for phototransduction and visual signal transmission 1. The enzyme is necessary for forming functional phosphodiesterase holoenzyme complexes and participates in retinal circadian rhythm photoentrainment via modulation of light-induced phase-shifts 1. Pathogenic variants in PDE6B cause autosomal recessive retinitis pigmentosa (RP), a progressive photoreceptor degeneration disease accounting for 2-5% of all RP cases 2. PDE6B mutations are particularly prevalent in early-onset RP, being the most common causative gene in patients experiencing initial symptoms within their first decade 3. The gene is also implicated in congenital stationary night blindness. Clinically, PDE6B-associated RP presents with classic features including attenuated retinal blood vessels, intraretinal pigmentation, and optic disc changes 2. Large genetic screening studies across Asian populations identified multiple pathogenic variants, including East Asian-specific variants 4. Emerging gene therapies show promise: prime editing systems targeting PDE6B nonsense mutations demonstrated 26.47% editing efficiency and protected rod cells from degeneration in preclinical models 5, while AAV5-mediated PDE6B gene delivery preserved photoreceptor structure and improved visual function in knockout rats 6.