Visual impairment in an optineurin mouse model of primary open-angle glaucoma.
Journal Article (Journal Article)
Primary open-angle glaucoma (POAG) is characterized by progressive neurodegeneration of retinal ganglion cells (RGCs). Why RGCs degenerate in low-pressure POAG remains poorly understood. To gain mechanistic insights, we developed a novel mouse model based on a mutation in human optineurin associated with hereditary, low-pressure POAG. This mouse improves the design and phenotype of currently available optineurin mice, which showed high global overexpression. Although both 18-month-old optineurin and nontransgenic control mice showed an age-related decrease in healthy axons and RGCs, the expression of mutant optineurin enhanced axonal degeneration and decreased RGC survival. Mouse visual function was determined using visual evoked potentials, which revealed specific visual impairment in contrast sensitivity. The E50K optineurin transgenic mouse described here exhibited clinical features of POAG and may be useful for mechanistic dissection of POAG and therapeutic development.
Full Text
Duke Authors
Cited Authors
- Tseng, HC; Riday, TT; McKee, C; Braine, CE; Bomze, H; Barak, I; Marean-Reardon, C; John, SWM; Philpot, BD; Ehlers, MD
Published Date
- June 2015
Published In
Volume / Issue
- 36 / 6
Start / End Page
- 2201 - 2212
PubMed ID
- 25818176
Pubmed Central ID
- PMC4433607
Electronic International Standard Serial Number (EISSN)
- 1558-1497
Digital Object Identifier (DOI)
- 10.1016/j.neurobiolaging.2015.02.012
Language
- eng
Conference Location
- United States