April 2009
Volume 50, Issue 13
Free
ARVO Annual Meeting Abstract  |   April 2009
Electroretinograms of the rd12 Mouse, a Model of Leber Congenital Amaurosis Caused by a Mutation in RPE65 Gene
Author Affiliations & Notes
  • X. Dai
    Ophthalmology, University of Florida, Gainesville, Florida
    Eye Hospital, Wenzhou Medical College, Wenzhou, China
  • B. Lei
    2Mason Eye Institute, Ophthalmology, Veterinary Medicine and Surgery, University of Missouri-Columbia, Columbia, Missouri
  • B. Chang
    The Jackson Laboratory, Bar Harbor, Maine
  • W. W. Hauswirth
    Ophthalmology, University of Florida, Gainesville, Florida
  • J. Qu
    Eye Hospital, Wenzhou Medical College, Wenzhou, China
  • J. Pang
    Ophthalmology, University of Florida, Gainesville, Florida
  • Footnotes
    Commercial Relationships  X. Dai, None; B. Lei, None; B. Chang, None; W.W. Hauswirth, AGTC, P; J. Qu, None; J. Pang, None.
  • Footnotes
    Support  NIH grants EY018331, EY13729, EY11123, NS36302, EY08571, and grants from the Macular Vision Research Foundation, Foundation Fighting Blindness, Juvenile Diabetes Research Foundation
Investigative Ophthalmology & Visual Science April 2009, Vol.50, 3600. doi:
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    • Get Citation

      X. Dai, B. Lei, B. Chang, W. W. Hauswirth, J. Qu, J. Pang; Electroretinograms of the rd12 Mouse, a Model of Leber Congenital Amaurosis Caused by a Mutation in RPE65 Gene. Invest. Ophthalmol. Vis. Sci. 2009;50(13):3600.

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

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Abstract

Purpose: : Leber Congenital Amaurosis (LCA) caused by RPE65 mutations leads to early onset of blindness. The rd12 mouse, a naturally occurring animal model of LCA with an Rpe65 mutation, exhibits residual photoreceptor functions which may be capable of translating into useful visual signals. To investigate the origin of those signals, normal C57BL/6J mice and rd12 mice at postnatal day 14 (P14) when both rod and cone morphologies are amlost normal were tested with flicker electroretinograms (ERGs) elicited at different stimulus frequencies and intensities.

Methods: : P14 C57BL/6J and rd12 mice were anesthetized and their pupils were dilated before recording. Dark-adapted ERGs were recorded with a serial of stimulation lights (intensity covers 4 log units). Dark- and light-adapted flicker ERGs were elicited with eight stimulus frequencies (5, 10, 15, 20, 25, 30, 35 and 40Hz) at two intensity levels. The dim stimulus (-3.0 Log cd·s·m-2) activates only rods and the bright stimulus (+0.5 Log cd·s·m-2) activates both rods and cones. Light-adapted ERGs were elicited under a steady background illumination (30 cd/m2).

Results: : In C57BL/6J mice with dim light stimuli, dark-adapted flicker ERGs were recorded with low frequency stimuli (5~20Hz), but not with high frequency stimuli (25~40Hz). With bright stimuli, flicker ERGs were observed at all eight frequencies. These cone-driven flicker ERG responses were confirmed in light-adapted normal mice with 5~40Hz stimuli. In rd12 mice with dim light stimuli, no dark-adapted flicker responses were seen at any of the eight frequencies. With bright stimuli, dark-adapted flicker responses were recorded at 5~20Hz, but not at 25~40Hz. With the same bright stimuli, light-adapted flicker ERGs were observed when the frequencies were at 5~20Hz, whereas no flicker signals were recorded at 25~40Hz.

Keywords: electroretinography: non-clinical • photoreceptors: visual performance • retinal degenerations: hereditary 
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