ZFIN ID: ZDB-PUB-980210-48
A dominant form of inherited retinal degeneration caused by a non-photoreceptor cell-specific mutation
Li, L. and Dowling, J.E.
Date: 1997
Source: Proceedings of the National Academy of Sciences of the United States of America   94: 11645-11650 (Journal)
Registered Authors: Dowling, John E., Li, Lei
Keywords: none
MeSH Terms:
  • Adaptation, Ocular
  • Aging
  • Animals
  • Embryo, Nonmammalian
  • Escape Reaction
  • Fish Diseases/genetics*
  • Genes, Dominant
  • Genetic Carrier Screening
  • Mutation*
  • Night Blindness/genetics
  • Night Blindness/physiopathology
  • Night Blindness/veterinary
  • Retina/growth & development
  • Retina/pathology
  • Retina/physiopathology*
  • Retinal Degeneration/genetics*
  • Retinal Degeneration/physiopathology
  • Retinal Degeneration/veterinary*
  • Sensory Thresholds
  • Visual Acuity
  • Zebrafish
PubMed: 9326664 Full text @ Proc. Natl. Acad. Sci. USA
FIGURES
ABSTRACT
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.
ADDITIONAL INFORMATION