Drusen, choroidal neovascularization, and retinal pigment epithelium dysfunction in SOD1-deficient mice: A model of age-related macular degeneration

• DOI: 10.1073/pnas.0602131103
• Publication Date: 2006-07-15T01:55:46Z
• Authors: Yutaka Imamura; Setsuko Noda; Kouhei Hashizume; Kei Shinoda; Mineko Yamaguchi; Satoshi Uchiyama; Takahiko Shimizu; Yutaka Mizushima; Takuji Shirasawa; Kazuo Tsubota
• Keywords: Drusen; Lipofuscin

Oxidative stress has long been linked to the pathogenesis of neurodegenerative diseases; however, whether it is a cause or merely consequence degenerative process still unknown. We show that mice deficient in Cu, Zn-superoxide dismutase (SOD1) have features typical age-related macular degeneration humans. Investigations senescent Sod1(-/-) different ages showed older animals had drusen, thickened Bruch's membrane, and choroidal neovascularization. The number drusen increased with age, exposure young excess light induced drusen. retinal pigment epithelial cells oxidative damage, their beta-catenin-mediated cellular integrity was disrupted, suggesting may affect junctional proteins necessary for barrier epithelium. These observations strongly suggest play causative role degeneration, our findings provide evidence free radical theory aging. In addition, these results demonstrate mouse valuable animal model study human degeneration.