Phototransduction in a Transgenic Mouse Model of Nougaret Night Blindness
نویسندگان
چکیده
منابع مشابه
Phototransduction in a transgenic mouse model of Nougaret night blindness.
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 sens...
متن کاملRod and cone function in the Nougaret form of stationary night blindness.
BACKGROUND Recently, a mutation (Gly38Asp) was identified in the alpha subunit of rod transducin in members of the Nougaret pedigree affected with dominantly inherited stationary night blindness. OBJECTIVE To evaluate retinal function in patients with the Gly38Asp gene defect. DESIGN Ocular examinations, including specialized measures of rod and cone function. SETTING A clinical research ...
متن کاملCongenital stationary night blindness: an animal model.
Electroretinographic studies of myctalopic Appaloosa horses demonstrated photopic and scotopic abnormalities similar to those in humans with congenital stationary night blindness (CSNB) of the Schubert-Bornschein type. The phototopic abnormalities consisted of reduced b-wave amplitudes and slower than normal b-wave implict time. The dark-adapted ERG's consisted of a simple negative potential; t...
متن کاملLrit3 Deficient Mouse (nob6): A Novel Model of Complete Congenital Stationary Night Blindness (cCSNB)
Mutations in LRIT3, coding for a Leucine-Rich Repeat, immunoglobulin-like and transmembrane domains 3 protein lead to autosomal recessive complete congenital stationary night blindness (cCSNB). The role of the corresponding protein in the ON-bipolar cell signaling cascade remains to be elucidated. Here we genetically and functionally characterize a commercially available Lrit3 knock-out mouse, ...
متن کاملA naturally occurring mouse model of X-linked congenital stationary night blindness.
PURPOSE To describe a naturally occurring X-linked recessive mutation, no b-wave (nob), that compromises visual transmission between photoreceptors and second-order neurons in mice. METHODS Affected mice were identified by recording the light-evoked response of the retina, the electroretinogram (ERG). To evaluate visual transmission, cortical potentials were recorded with a scalp electrode. T...
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ژورنال
عنوان ژورنال: Journal of Neuroscience
سال: 2006
ISSN: 0270-6474,1529-2401
DOI: 10.1523/jneurosci.1322-06.2006