Phototransduction in a Transgenic Mouse Model of Nougaret Night Blindness

Overview

Journal J Neurosci

Specialty Neurology

Date 2006 Jun 24

PMID 16793893

Citations 14

Authors

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

Affiliations

Soon will be listed here.

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.

Citing Articles

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