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The Journal of Neuroscience, June 21, 2006, 26(25):6863-6872; doi:10.1523/JNEUROSCI.1322-06.2006

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Cellular/Molecular
Phototransduction in a Transgenic Mouse Model of Nougaret Night Blindness

Mustapha Moussaif,¹ William W. Rubin,² Vasily Kerov,¹ Rebecca Reh,³ Desheng Chen,⁴ Janis Lem,⁵ Ching-Kang Chen,⁴ James B. Hurley,³ Marie E. Burns,² and Nikolai O. Artemyev¹

¹Department of Physiology and Biophysics, University of Iowa College of Medicine, Iowa City, Iowa 52242, ²Center for Neuroscience and Department of Psychiatry and Behavioral Sciences, University of California–Davis, Davis, California 95616, ³Department of Biochemistry, University of Washington School of Medicine, Seattle, Washington 98195, ⁴Department of Biochemistry, Virginia Commonwealth University, Richmond, Virginia 23298, and ⁵Department of Ophthalmology, Program in Genetics, and Tufts Center for Vision Research, Tufts University School of Medicine, Boston, Massachusetts 02111

Correspondence should be addressed to Dr. Nikolai O. Artemyev, Department of Physiology and Biophysics, University of Iowa College of Medicine, 5-532 Bowen Science Building, 51 Newton Road, Iowa City, IA 52242-1109. Email: nikolai-artemyev{at}uiowa.edu

The Nougaret form of dominant stationary night blindness is linked to a G38D mutation in the rod transducin- subunit (T). In this study, we have examined the mechanism of Nougaret night blindness using transgenic mice expressing TG38D. The biochemical, electrophysiological, and vision-dependent behavioral analyses of the mouse model revealed a unique phenotype of reduced rod sensitivity, impaired activation, and slowed recovery of the phototransduction cascade. Two key deficiencies in TG38D function, its poor ability to activate PDE6 (cGMP phosphodiesterase) and decreased GTPase activity, are found to be the major mechanisms altering visual signaling in transgenic mice. Despite these defects, rod-mediated sensitivity in heterozygous mice is not decreased to the extent seen in heterozygous Nougaret patients.

Key words: photoreceptor; phototransduction; transducin; retina; G-proteins; night blindness

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Received March 28, 2006; revised May 9, 2006; accepted May 11, 2006.

Correspondence should be addressed to Dr. Nikolai O. Artemyev, Department of Physiology and Biophysics, University of Iowa College of Medicine, 5-532 Bowen Science Building, 51 Newton Road, Iowa City, IA 52242-1109. Email: nikolai-artemyev{at}uiowa.edu

This article has been cited by other articles:

				V. Kerov, W. W. Rubin, M. Natochin, N. A. Melling, M. E. Burns, and N. O. Artemyev N-Terminal Fatty Acylation of Transducin Profoundly Influences Its Localization and the Kinetics of Photoresponse in Rods J. Neurosci., September 19, 2007; 27(38): 10270 - 10277. [Abstract] [Full Text] [PDF]

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