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.
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.
Authors: Miranda L Scalabrino; Sanford L Boye; Kathryn M H Fransen; Jennifer M Noel; Frank M Dyka; Seok Hong Min; Qing Ruan; Charles N De Leeuw; Elizabeth M Simpson; Ronald G Gregg; Maureen A McCall; Neal S Peachey; Shannon E Boye Journal: Hum Mol Genet Date: 2015-08-26 Impact factor: 6.150
Authors: Marion Neuillé; Catherine W Morgans; Yan Cao; Elise Orhan; Christelle Michiels; José-Alain Sahel; Isabelle Audo; Robert M Duvoisin; Kirill A Martemyanov; Christina Zeitz Journal: Eur J Neurosci Date: 2015-07-04 Impact factor: 3.386
Authors: M Joseph Phillips; Sarah Webb-Wood; Amanda E Faulkner; Seema B Jabbar; Valerie Biousse; Nancy J Newman; Vi T Do; Jeffrey H Boatright; Douglas C Wallace; Machelle T Pardue Journal: Invest Ophthalmol Vis Sci Date: 2010-07-29 Impact factor: 4.799
Authors: Eldon E Geisert; Lu Lu; Natalie E Freeman-Anderson; Justin P Templeton; Mohamed Nassr; Xusheng Wang; Weikuan Gu; Yan Jiao; Robert W Williams Journal: Mol Vis Date: 2009-08-31 Impact factor: 2.367