Visual impairment in an optineurin mouse model of primary open-angle glaucoma.

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

Electronic International Standard Serial Number (EISSN)

  • 1558-1497

International Standard Serial Number (ISSN)

  • 0197-4580

Digital Object Identifier (DOI)

  • 10.1016/j.neurobiolaging.2015.02.012

Language

  • eng