Phototransduction in a transgenic mouse model of Nougaret night blindness

Mustapha Moussaif; William W Rubin; Vasily Kerov; Rebecca Reh; Desheng Chen; Janis Lem; Ching-Kang Chen; James B Hurley; Marie E Burns; Nikolai O Artemyev

doi:10.1523/JNEUROSCI.1322-06.2006

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Phototransduction in a transgenic mouse model of Nougaret night blindness

Journal article

Open access

Peer reviewed

Phototransduction in a transgenic mouse model of Nougaret night blindness

Mustapha Moussaif, William W Rubin, Vasily Kerov, Rebecca Reh, Desheng Chen, Janis Lem, Ching-Kang Chen, James B Hurley, Marie E Burns and Nikolai O Artemyev

The Journal of neuroscience, Vol.26(25), pp.6863-6872

06/21/2006

DOI: 10.1523/JNEUROSCI.1322-06.2006

PMCID: PMC6673833

PMID: 16793893

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https://doi.org/10.1523/JNEUROSCI.1322-06.2006View

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Abstract

The Nougaret form of dominant stationary night blindness is linked to a G38D mutation in the rod transducin-alpha subunit (Talpha). In this study, we have examined the mechanism of Nougaret night blindness using transgenic mice expressing TalphaG38D. 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 TalphaG38D 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.

Electroretinography - methods

Retina - metabolism

Membrane Potentials - radiation effects

Gene Expression - physiology

Night Blindness - pathology

Night Blindness - genetics

Disease Models, Animal

Membrane Potentials - drug effects

Transducin - genetics

Blotting, Western - methods

Mice, Inbred C57BL

Retina - physiopathology

Dark Adaptation - physiology

Mice, Transgenic

Membrane Potentials - physiology

Immunohistochemistry - methods

Vision, Ocular - genetics

Guanosine 5'-O-(3-Thiotriphosphate) - metabolism

Animals

Eye Proteins - metabolism

Photic Stimulation - methods

Sensory Thresholds - physiology

Night Blindness - physiopathology

Mice

Retina - pathology

Retinal Rod Photoreceptor Cells - physiopathology

Details

Title: Subtitle: Phototransduction in a transgenic mouse model of Nougaret night blindness
Creators: Mustapha Moussaif - Department of Physiology and Biophysics, University of Iowa College of Medicine, Iowa City, Iowa 52242, USA
William W Rubin
Vasily Kerov
Rebecca Reh
Desheng Chen
Janis Lem
Ching-Kang Chen
James B Hurley
Marie E Burns
Nikolai O Artemyev
Resource Type: Journal article
Publication Details: The Journal of neuroscience, Vol.26(25), pp.6863-6872
Publisher: United States
DOI: 10.1523/JNEUROSCI.1322-06.2006
PMID: 16793893
PMCID: PMC6673833
ISSN: 0270-6474
eISSN: 1529-2401
Grant note: R01 EY006641 / NEI NIH HHS EY10843 / NEI NIH HHS R01 EY014047 / NEI NIH HHS EY14047 / NEI NIH HHS R01 EY12682 / NEI NIH HHS EY06641 / NEI NIH HHS R01 EY014047-05 / NEI NIH HHS EY13811 / NEI NIH HHS
Language: English
Date published: 06/21/2006
Academic Unit: Molecular Physiology and Biophysics; Iowa Neuroscience Institute; Ophthalmology and Visual Sciences
Record Identifier: 9984070879602771

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