title: Spontaneous occurrence of a potentially night blinding disorder in guinea pigs.
abstract: Several hereditary retinal disorders such as retinitis pigmentosa and congenital stationary night blindness compromise, sometimes exclusively, the activity of the rod pathway. Unfortunately, there are few animal models of these disorders that could help us better understand the pathophysiological processes involved. The purpose of this report is to present a pedigree of guinea pigs where, as a result of a consanguineous mating and subsequent selective breeding, we developed a new and naturally occurring animal model of a rod disorder. Analysis of the retinal function with the electroretinogram reveals that the threshold for rod-mediated electroretinograms (ERGs) is significantly increased by more than 2 log-units compared to that of normal guinea pigs. Furthermore, in response to a suprathreshold stimulus, also delivered under scotopic condition, which yield a mixed cone-rod response in normal guinea pigs, the ERG waveform in our mutant guinea pigs is almost identical (amplitude and timing of a- and b-waves) to that evoked in photopic condition. The above would thus suggest either a structural (abnormal development or absence) or a functional deficiency of the rod photoreceptors. We believe that our pedigree possibly represents a new animal model of a night blinding disorder, and that this condition is inherited as anautosomal recessive trait in the guinea pig population.
authors.affiliation: Departments of Ophthalmology and Neurology-Neurosurgery, McGill University-Montreal Children's Hospital Research Institute, Montréal, Québec, Canada.
authors.forename: Julie
authors.initials: J
authors.lastname: Racine
authors.forename: Darren
authors.initials: D
authors.lastname: Behn
authors.forename: Eric
authors.initials: E
authors.lastname: Simard
authors.forename: Pierre
authors.initials: P
authors.lastname: Lachapelle
mesh_terms: D000818:Animals; D017849:Animals, Inbred Strains; D003623:Dark Adaptation; D004195:Disease Models, Animal; D004596:Electroretinography; D006168:Guinea Pigs; D007178:Inbreeding; D009755:Night Blindness / Q000235:genetics* / Q000503:physiopathology; D010375:Pedigree; D010775:Photic Stimulation; D017948:Retinal Rod Photoreceptor Cells / Q000503:physiopathology; D012376:Rodent Diseases / Q000235:genetics*