July 2019
Volume 60, Issue 9
Open Access
ARVO Annual Meeting Abstract  |   July 2019
PRCD supports the organized structure of the photoreceptor outer segment
Author Affiliations & Notes
  • WILLIAM SPENCER
    Duke University, Durham, North Carolina, United States
  • Jillian Nydam Pearring
    University of Michigan, Michigan, United States
  • Jindong Ding
    Duke University, Durham, North Carolina, United States
  • Nikolai P Skiba
    Duke University, Durham, North Carolina, United States
  • Marie E Burns
    University of California Davis, California, United States
  • Vadim Y Arshavsky
    Duke University, Durham, North Carolina, United States
  • Footnotes
    Commercial Relationships   WILLIAM SPENCER, None; Jillian Pearring, None; Jindong Ding, None; Nikolai Skiba, None; Marie Burns, None; Vadim Arshavsky, None
  • Footnotes
    Support  None
Investigative Ophthalmology & Visual Science July 2019, Vol.60, 6052. doi:
  • Views
  • Share
  • Tools
    • Alerts
      ×
      This feature is available to authenticated users only.
      Sign In or Create an Account ×
    • Get Citation

      WILLIAM SPENCER, Jillian Nydam Pearring, Jindong Ding, Nikolai P Skiba, Marie E Burns, Vadim Y Arshavsky; PRCD supports the organized structure of the photoreceptor outer segment. Invest. Ophthalmol. Vis. Sci. 2019;60(9):6052.

      Download citation file:


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

      ×
  • Supplements
Abstract

Purpose : PRCD is a small photoreceptor disc specific protein whose C2Y mutation is a common cause of blinding canine retinal disease called progressive rod-cone degeneration (prcd). This exact mutation, and several others have been identified in human patients diagnosed with retinitis pigmentosa. After we showed that this point mutation completely mislocalizes the protein from discs, and that PRCD is a rhodopsin binding protein, we generated a PRCD knockout mouse to ascertain its function in the outer segment.

Methods : PRCD knockout mice were generated and their retinas analyzed by electron microscopy, electroretinogram, immunofluorescence staining, and Western blotting.

Results : PRCD knockout mouse photoreceptors degenerate at a similar rate as dogs containing a C2Y mutation in the protein. As evident by electron microscopy, the outer segments from these mice have a structural defect, and many of them are completely disorganized. This pheotype persists despite normal single rod photoresponses and normal localization and abundance of all tested outer segment proteins.

Conclusions : PRCD is essential for the highly organized structure of the photoreceptor outer segment.

This abstract was presented at the 2019 ARVO Annual Meeting, held in Vancouver, Canada, April 28 - May 2, 2019.

×
×

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.

×