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Publication : A naturally occurring mouse model of X-linked congenital stationary night blindness.

First Author  Pardue MT Year  1998
Journal  Invest Ophthalmol Vis Sci Volume  39
Issue  12 Pages  2443-9
PubMed ID  9804152 Mgi Jnum  J:50824
Mgi Id  MGI:1309958 Citation  Pardue MT, et al. (1998) A naturally occurring mouse model of X-linked congenital stationary night blindness. Invest Ophthalmol Vis Sci 39(12):2443-9
abstractText  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. The inheritance pattern. For nob was defined by breeding nob animals with normal mice. Retinal histologic analysis was performed by light microscopy. RESULTS. Although the photoreceptor-mediated ERG component (a-wave) was normal in nob mice, the major response component reflecting postreceptoral neuronal activity (b- wave) was missing. Visually-driven cortical activity was also abnormal in nob animals. At the light microscopic level, the nob retina appeared to have a normal cytoarchitecture. CONCLUSIONS. These findings suggest that the nob defect interferes with the transmission of visual information through the retina and that these mice are a useful model for the study of outer retinal synaptic function. In addition, this mutant mouse seems to provide an animal model for the complete form of congenital stationary night blindness, a human disorder in which patients have a profound loss of rod-mediated visual sensitivity.
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