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Related Experiment Videos

9-cis Retinal increased in retina of RPE65 knockout mice with decrease in coat pigmentation.

Jie Fan1, Bill X Wu, Tadeusz Sarna

  • 1Department of Ophthalmology, Medical University of South Carolina, Charleston, SC, USA. fan@musc.edu

Photochemistry and Photobiology
|March 24, 2006
PubMed
Summary
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RPE65 protein is crucial for vision. Rpe65 knockout mice have altered visual pigment formation, with tan mice showing better responses due to increased 9-cis retinal and pigment levels.

Area of Science:

  • Biochemistry
  • Vision Science
  • Genetics

Background:

  • Retinal pigment epithelium protein 65 (RPE65) is vital for visual pigment chromophore regeneration.
  • Rpe65 knockout mice exhibit minimal visual response due to 9-cis retinal forming isorhodopsin.
  • Coat color variations (agouti vs. tan) in Rpe65 knockout mice influence visual responses.

Purpose of the Study:

  • To investigate the impact of RPE65 deficiency on visual pigment formation and photoreceptor health.
  • To explore the role of coat color in modulating visual function in Rpe65 knockout mice.
  • To determine the correlation between pigment levels, opsin activity, and photoreceptor degeneration.

Main Methods:

  • Generation and characterization of Rpe65 knockout mice with different coat colors.

Related Experiment Videos

  • Electroretinography to measure visual responses.
  • Analysis of visual pigment levels (isorhodopsin) and opsin levels under different light conditions (dark-rearing vs. cyclic light).

    • Main Results:

    • Rpe65 knockout mice accumulate isorhodopsin, particularly with prolonged dark-rearing.
    • Tan Rpe65 knockout mice display enhanced visual responses due to higher 9-cis retinal and isorhodopsin levels compared to agouti mice.
    • Photoreceptor degeneration is reduced in dark-reared animals and correlates with lower levels of free opsin, suggesting opsin activity contributes to degeneration.

    Conclusions:

    • RPE65 is essential for regenerating the native visual chromophore, but alternative pathways involving 9-cis retinal can support some visual function.
    • Coat color acts as a modifier of visual response and photoreceptor survival in Rpe65 deficiency.
    • Free opsin accumulation, rather than pigment regeneration itself, appears to drive photoreceptor degeneration in Rpe65 knockout models.