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. |