A dominant form of inherited retinal degeneration caused by a non-photoreceptor cell-specific mutation.
We have isolated a dominant mutation, night blindness a (nba), that causes a slow retinal degeneration in zebrafish. Heterozygous nba fish have normal vision through 2-3 months of age but subsequently become night blind. By 9.5 months of age, visual sensitivity of affected fish may be decreased more than two log units, or 100-fold, as measured behaviorally. Electroretinographic (ERG) thresholds of mutant fish are also raised significantly, and the ERG b-wave shows a delayed implicit time. These defects are due primarily to a late-onset photoreceptor cell degeneration involving initially the rods but eventually the cones as well. Homozygous nba fish display an early-onset neuronal degeneration throughout the retina and elsewhere in the central nervous system. As a result, animals develop with small eyes and die by 4-5 days postfertilization (pf). These latter data indicate that the mutation affecting nba fish is not in a photoreceptor cell-specific gene.
Li
L
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Department of Molecular and Cellular Biology, Harvard University, 16 Divinity Avenue, Cambridge, MA 02138, USA.
Dowling
J E
JE
D000221:Adaptation, Ocular; D000375:Aging; D000818:Animals; D004625:Embryo, Nonmammalian; D004924:Escape Reaction; D005393:Fish Diseases / Q000235:genetics*; D005799:Genes, Dominant; D006580:Genetic Carrier Screening; D009154:Mutation; D009755:Night Blindness / Q000235:genetics / Q000503:physiopathology / Q000662:veterinary; D012160:Retina / Q000254:growth & development / Q000473:pathology / Q000503:physiopathology*; D012162:Retinal Degeneration / Q000235:genetics* / Q000503:physiopathology / Q000662:veterinary*; D012684:Sensory Thresholds; D014792:Visual Acuity; D015027:Zebrafish