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Previous Article | Next Article
The Journal of Neuroscience, July 15, 1999, 19(14):5889-5897
Disruption of a Retinal Guanylyl Cyclase Gene Leads to Cone-Specific Dystrophy and Paradoxical Rod Behavior
Ruey-Bing Yang2, Susan W. Robinson1, Wei-Hong Xiong5, King-Wai Yau5, David G. Birch3, 4, and David L. Garbers1, 2 1 Howard Hughes Medical Institute and Departments of 2 Pharmacology and 3 Ophthalmology, University of Texas Southwestern Medical Center, Dallas, Texas 75235-9050, 4 Retina Foundation of the Southwest, Dallas, Texas 75231, and 5 Howard Hughes Medical Institute and Department of Neuroscience, Johns Hopkins University School of Medicine, Baltimore, Maryland 21205
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 of the second retinal cyclase (GC-F) as well as the numbers and morphology of rods remained unchanged in GC-E null mice. However, rods isolated from such mice, despite having a normal dark current, recovered from a light flash markedly faster. Unexpectedly, the a- and b-waves of electroretinograms (ERG) from dark-adapted null mice were suppressed markedly. Cones, initially present in normal numbers in the retina, disappeared by 5 weeks, based on ERG and histology. Thus, the GC-E-deficient mouse defines a model for cone dystrophy, but it also demonstrates that morphologically normal rods display paradoxical behavior in their responses to light.
Key words: guanylyl cyclases; retina; photoreceptors; gene disruption; cone dystrophy; mice; cyclic GMP; guanylyl cyclase-E
Copyright © 1999 Society for Neuroscience 0270-6474/99/19145889-09$05.00/0
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