Disruption of a Retinal Guanylyl Cyclase Gene Leads to Cone-Specific Dystrophy and Paradoxical Rod Behavior

摘要: One of two orphan photoreceptor guanylyl cyclases that are highly conserved from fish to mammals, GC-E (or retGC1) was eliminated by gene disruption. Expression the second retinal cyclase (GC-F) as well numbers and morphology rods remained unchanged in null mice. However, isolated such mice, despite having a normal dark current, recovered light flash markedly faster. Unexpectedly, a- b-waves electroretinograms (ERG) dark-adapted mice were suppressed markedly. Cones, initially present retina, disappeared 5 weeks, based on ERG histology. Thus, GC-E-deficient mouse defines model for cone dystrophy, but it also demonstrates morphologically display paradoxical behavior their responses light.