title: A naturally occurring mouse model of X-linked congenital stationary night blindness.
abstract: OBJECTIVE
  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.affiliation: Research Service, Hines VA Hospital, Illinois 60141, USA.
authors.forename: M T
authors.initials: MT
authors.lastname: Pardue
authors.forename: M A
authors.initials: MA
authors.lastname: McCall
authors.forename: M M
authors.initials: MM
authors.lastname: LaVail
authors.forename: R G
authors.initials: RG
authors.lastname: Gregg
authors.forename: N S
authors.initials: NS
authors.lastname: Peachey
mesh_terms: D000818:Animals; D003623:Dark Adaptation; D004195:Disease Models, Animal; D004596:Electroretinography; D005074:Evoked Potentials, Visual / Q000502:physiology*; D005260:Female; D008040:Genetic Linkage; D008027:Light; D008297:Male; D051379:Mice; D008807:Mice, Inbred BALB C; D008817:Mice, Mutant Strains; D009154:Mutation; D009474:Neurons / Q000473:pathology / Q000502:physiology; D009755:Night Blindness / Q000235:genetics* / Q000473:pathology / Q000503:physiopathology; D010375:Pedigree; D017956:Photoreceptor Cells, Invertebrate / Q000473:pathology / Q000503:physiopathology; D014785:Vision, Ocular; D014960:X Chromosome / Q000235:genetics*