Investigative Ophthalmology & Visual Science Cover Image for Volume 57, Issue 12
September 2016
Volume 57, Issue 12
Open Access
ARVO Annual Meeting Abstract  |   September 2016
Double Knockout of Centrin 2 and 3 Causes Photoreceptor Degeneration in Mouse
Author Affiliations & Notes
  • Guoxin Ying
    Ophthalmology, University of Utah, Salt Lake City, Utah, United States
  • Jeanne M Frederick
    Ophthalmology, University of Utah, Salt Lake City, Utah, United States
  • Wolfgang Baehr
    Ophthalmology, University of Utah, Salt Lake City, Utah, United States
  • Footnotes
    Commercial Relationships   Guoxin Ying, None; Jeanne Frederick, None; Wolfgang Baehr, None
  • Footnotes
    Support  EY08123, EY019298, EY014800-039003 (NEI core grant); RPB Nelson Trust
Investigative Ophthalmology & Visual Science September 2016, Vol.57, 175. doi:
  • Views
  • Share
  • Tools
    • Alerts
      ×
      This feature is available to authenticated users only.
      Sign In or Create an Account ×
    • Get Citation

      Guoxin Ying, Jeanne M Frederick, Wolfgang Baehr; Double Knockout of Centrin 2 and 3 Causes Photoreceptor Degeneration in Mouse. Invest. Ophthalmol. Vis. Sci. 2016;57(12):175.

      Download citation file:


      © ARVO (1962-2015); The Authors (2016-present)

      ×
  • Supplements
Abstract

Purpose : The mouse genome contains four centrin genes (Cetn1-4), which encode four conserved basal body (bb)/cilium-associated calcium-binding protein centrins. We have previously generated Cetn1 and Cetn2 knockout (KO) with no detectable retina phenotype (Avasthi et al., 2013) (Ying et al., 2014). This study continues to examine the role of Cetn 2 and Cetn3 in mouse photoreceptor development and maintenance.

Methods : We generated a Cetn3 knockout (KO) mouse using Eucomm ES cell lines and subsequently Cetn2 and 3 double kockout (dKO) mice by crossing Cetn3 KO with Cetn2 KO. Electroretinography (ERG) was used to assess the function of mutant retina, and immunostaining was used to check the ciliary trafficking of rhodopsin and other major outer segment (OS) phototransduction proteins.

Results : Cetn3 KO mouse has normal ERG and retinal morphology up to 10 month old, and rhodopsin and other major outer segment (OS) phototransduction proteins traffic correctly. However, Cetn2/Cetn3 dKO mouse has severely reduced ERG, shortened photoreceptor OS, and thinned outer nuclear layer (ONL). Rhodopsin and other OS proteins traffic normally to the outer segments at 3 month of age.

Conclusions : Cetn2 deletion is associated with dysosmia and trafficking in mouse olfactory neurons. Cetn2 and Cetn3 single knockouts have no recognizable retina phenotype suggesting that Cetn2 and Cetn3 are redundant in retina. However, both Cetn2 and Cetn3 are required for photoreceptor survival.

This is an abstract that was submitted for the 2016 ARVO Annual Meeting, held in Seattle, Wash., May 1-5, 2016.

×
×

This PDF is available to Subscribers Only

Sign in or purchase a subscription to access this content. ×

You must be signed into an individual account to use this feature.

×