Functional abnormalities in the retinal pigment epithelium of CFTR mutant mice

Jiang Wu, Alan D Marmorstein, Neal S. Peachey

Research output: Contribution to journalArticle

18 Citations (Scopus)

Abstract

In response to light, the mouse retinal pigment epithelium (RPE) generates a series of slow changes in potential that are referred to as the c-wave, fast oscillation (FO) and light peak (LP) of the electroretinogram (ERG). While the FO is known to reflect a Cl- conductance generated at the basal membrane of the RPE, the specific channel (s) underlying this potential has not been identified. In the present study we examined two strains of mice with cftr mutations to define the contribution that cystic fibrosis transmembrane regulator (CFTR)-mediated Cl- conductance makes to the mouse ERG. Responses obtained from cftrΔ508/Δ508 mice exhibited an overall reduction in all components generated by the RPE in response to light without alteration of the luminance response function. Responses obtained from cftr-/- mice were also reduced in amplitude. These results illustrate the usefulness of ERG analysis of mice deficient in ion channels that are expressed in the RPE, and indicate that CFTR contributes to the generation of RPE-driven ERG components, but that it is not the sole generator of any one of these components.

Original languageEnglish (US)
Pages (from-to)424-428
Number of pages5
JournalExperimental Eye Research
Volume83
Issue number2
DOIs
StatePublished - Aug 2006
Externally publishedYes

Fingerprint

Retinal Pigment Epithelium
Cystic Fibrosis
Light
Ion Channels
Mutation
Membranes

Keywords

  • cystic fibrosis transmembrane regulator
  • electroretinogram
  • fast oscillation
  • mouse
  • retinal pigment epithelium

ASJC Scopus subject areas

  • Ophthalmology
  • Sensory Systems

Cite this

Functional abnormalities in the retinal pigment epithelium of CFTR mutant mice. / Wu, Jiang; Marmorstein, Alan D; Peachey, Neal S.

In: Experimental Eye Research, Vol. 83, No. 2, 08.2006, p. 424-428.

Research output: Contribution to journalArticle

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