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
Language
- eng
Conference Location
- United States