Retinal rod photoreceptor-specific gene mutation perturbs cone pathway development.

Journal Article (Journal Article)

Rod-specific photoreceptor dystrophies are complicated by the delayed death of genetically normal neighboring cones. In transgenic (Tg) swine with a rod-specific (rhodopsin) gene mutation, cone photoreceptor physiology was normal for months but later declined, consistent with delayed cone cell death. Surprisingly, cone postreceptoral function was markedly abnormal when cone photoreceptor physiology was still normal. The defect was localized to hyperpolarizing cells postsynaptic to the middle wavelength-sensitive cones. Recordings throughout postnatal development indicated a failure of cone circuitry maturation, a novel mechanism of secondary cone abnormality in rod dystrophy. The results have implications for therapy for human retinal dystrophies and raise the possibility that rod afferent activity plays a role in the postnatal maturation of cone retinal circuitry.

Full Text

Duke Authors

Cited Authors

  • Banin, E; Cideciyan, AV; Alemán, TS; Petters, RM; Wong, F; Milam, AH; Jacobson, SG

Published Date

  • July 1999

Published In

Volume / Issue

  • 23 / 3

Start / End Page

  • 549 - 557

PubMed ID

  • 10433266

International Standard Serial Number (ISSN)

  • 0896-6273

Digital Object Identifier (DOI)

  • 10.1016/s0896-6273(00)80807-7


  • eng

Conference Location

  • United States