 |
||||
|
||||
|
||||
|
||||
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
This article has been cited by other articles:
H. Lin, C.-F. Cheng, H.-H. Hou, W.-S. Lian, Y.-C. Chao, Y.-Y. Ciou, B. Djoko, M.-T. Tsai, C.-J. Cheng, and R.-B. Yang
Disruption of Guanylyl Cyclase-G Protects against Acute Renal Injury
J. Am. Soc. Nephrol., February 1, 2008; 19(2): 339 - 348.
[Abstract] [Full Text] [PDF]
G. Stearns, M. Evangelista, J. M. Fadool, and S. E. Brockerhoff
A Mutation in the Cone-Specific pde6 Gene Causes Rapid Cone Photoreceptor Degeneration in Zebrafish
J. Neurosci., December 12, 2007; 27(50): 13866 - 13874.
[Abstract] [Full Text] [PDF]
W. Baehr, S. Karan, T. Maeda, D.-G. Luo, S. Li, J. D. Bronson, C. B. Watt, K.-W. Yau, J. M. Frederick, and K. Palczewski
The Function of Guanylate Cyclase 1 and Guanylate Cyclase 2 in Rod and Cone Photoreceptors
J. Biol. Chem., March 23, 2007; 282(12): 8837 - 8847.
[Abstract] [Full Text] [PDF]
C. Punzo and C. Cepko
Cellular Responses to Photoreceptor Death in the rd1 Mouse Model of Retinal Degeneration
Invest. Ophthalmol. Vis. Sci., February 1, 2007; 48(2): 849 - 857.
[Abstract] [Full Text] [PDF]
B. Chang, M. S. Dacey, N. L. Hawes, P. F. Hitchcock, A. H. Milam, P. Atmaca-Sonmez, S. Nusinowitz, and J. R. Heckenlively
Cone Photoreceptor Function Loss-3, a Novel Mouse Model of Achromatopsia Due to a Mutation in Gnat2
Invest. Ophthalmol. Vis. Sci., November 1, 2006; 47(11): 5017 - 5021.
[Abstract] [Full Text] [PDF]
S. E. Haire, J. Pang, S. L. Boye, I. Sokal, C. M. Craft, K. Palczewski, W. W. Hauswirth, and S. L. Semple-Rowland
Light-Driven Cone Arrestin Translocation in Cones of Postnatal Guanylate Cyclase-1 Knockout Mouse Retina Treated with AAV-GC1.
Invest. Ophthalmol. Vis. Sci., September 1, 2006; 47(9): 3745 - 3753.
[Abstract] [Full Text] [PDF]
D.-G. Luo and K.-W. Yau
Rod Sensitivity of Neonatal Mouse and Rat
J. Gen. Physiol., August 29, 2005; 126(3): 263 - 269.
[Abstract] [Full Text] [PDF]
I. Sokal, W. J. Dupps, M. A. Grassi, J. Brown Jr, L. M. Affatigato, N. Roychowdhury, L. Yang, S. Filipek, K. Palczewski, E. M. Stone, et al.
A Novel GCAP1 Missense Mutation (L151F) in a Large Family with Autosomal Dominant Cone-Rod Dystrophy (adCORD)
Invest. Ophthalmol. Vis. Sci., April 1, 2005; 46(4): 1124 - 1132.
[Abstract] [Full Text] [PDF]
S. Huttl, S. Michalakis, M. Seeliger, D.-G. Luo, N. Acar, H. Geiger, K. Hudl, R. Mader, S. Haverkamp, M. Moser, et al.
Impaired Channel Targeting and Retinal Degeneration in Mice Lacking the Cyclic Nucleotide-Gated Channel Subunit CNGB1
J. Neurosci., January 5, 2005; 25(1): 130 - 138.
[Abstract] [Full Text] [PDF]
J. E. Coleman and S. L. Semple-Rowland
GC1 Deletion Prevents Light-Dependent Arrestin Translocation in Mouse Cone Photoreceptor Cells
Invest. Ophthalmol. Vis. Sci., January 1, 2005; 46(1): 12 - 16.
[Abstract] [Full Text] [PDF]
S. Xu, Y. Wang, H. Zhao, L. Zhang, W. Xiong, K.-W. Yau, H. Hiel, E. Glowatzki, D. K. Ryugo, and D. Valle
PHR1, a PH Domain-Containing Protein Expressed in Primary Sensory Neurons
Mol. Cell. Biol., October 15, 2004; 24(20): 9137 - 9151.
[Abstract] [Full Text] [PDF]
J. E. Coleman, Y. Zhang, G. A. J. Brown, and S. L. Semple-Rowland
Cone Cell Survival and Downregulation of GCAP1 Protein in the Retinas of GC1 Knockout Mice
Invest. Ophthalmol. Vis. Sci., October 1, 2004; 45(10): 3397 - 3403.
[Abstract] [Full Text] [PDF]
M. Kuhn
Structure, Regulation, and Function of Mammalian Membrane Guanylyl Cyclase Receptors, With a Focus on Guanylyl Cyclase-A
Circ. Res., October 17, 2003; 93(8): 700 - 709.
[Abstract] [Full Text] [PDF]
M. E. Pennesi, K. A. Howes, W. Baehr, and S. M. Wu
Guanylate cyclase-activating protein (GCAP) 1 rescues cone recovery kinetics in GCAP1/GCAP2 knockout mice
PNAS, May 27, 2003; 100(11): 6783 - 6788.
[Abstract] [Full Text] [PDF]
R. K. Koenekoop, G. A. Fishman, A. Iannaccone, H. Ezzeldin, M. L. Ciccarelli, A. Baldi, J. S. Sunness, A. J. Lotery, M. M. Jablonski, S. J. Pittler, et al.
Electroretinographic Abnormalities in Parents of Patients With Leber Congenital Amaurosis Who Have Heterozygous GUCY2D Mutations
Arch Ophthalmol, October 1, 2002; 120(10): 1325 - 1330.
[Abstract] [Full Text] [PDF]
A. Mendez, M. E. Burns, I. Sokal, A. M. Dizhoor, W. Baehr, K. Palczewski, D. A. Baylor, and J. Chen
Role of guanylate cyclase-activating proteins (GCAPs) in setting the flash sensitivity of rod photoreceptors
PNAS, August 1, 2001; (2001) 171308998.
[Abstract] [Full Text] [PDF]
K. A. Lucas, G. M. Pitari, S. Kazerounian, I. Ruiz-Stewart, J. Park, S. Schulz, K. P. Chepenik, and S. A. Waldman
Guanylyl Cyclases and Signaling by Cyclic GMP
Pharmacol. Rev., September 1, 2000; 52(3): 375 - 414.
[Abstract] [Full Text] [PDF]
F HAFEZI, C GRIMM, B C SIMMEN, A WENZEL, and C E REMÉ
Molecular ophthalmology: an update on animal models for retinal degenerations and dystrophies
Br. J. Ophthalmol., August 1, 2000; 84(8): 922 - 927.
[Full Text]
C. A. G. G. Driessen, H. J. Winkens, K. Hoffmann, L. D. Kuhlmann, B. P. M. Janssen, A. H. M. Van Vugt, J. P. Van Hooser, B. E. Wieringa, A. F. Deutman, K. Palczewski, et al.
Disruption of the 11-cis-Retinol Dehydrogenase Gene Leads to Accumulation of cis-Retinols and cis-Retinyl Esters
Mol. Cell. Biol., June 15, 2000; 20(12): 4275 - 4287.
[Abstract] [Full Text]
V. Ramamurthy, C. Tucker, S. E. Wilkie, V. Daggett, D. M. Hunt, and J. B. Hurley
Interactions within the Coiled-coil Domain of RetGC-1 Guanylyl Cyclase Are Optimized for Regulation Rather than for High Affinity
J. Biol. Chem., July 6, 2001; 276(28): 26218 - 26229.
[Abstract] [Full Text] [PDF]
A. Mendez, M. E. Burns, I. Sokal, A. M. Dizhoor, W. Baehr, K. Palczewski, D. A. Baylor, and J. Chen
Role of guanylate cyclase-activating proteins (GCAPs) in setting the flash sensitivity of rod photoreceptors
PNAS, August 14, 2001; 98(17): 9948 - 9953.
[Abstract] [Full Text] [PDF]
|
|
|
|
 |
|