Original Research Article
N-acetylcysteine promotes long-term survival of cones in a model of retinitis pigmentosa
Article first published online: 19 APR 2011
Copyright © 2010 Wiley-Liss, Inc.
Journal of Cellular Physiology
Volume 226, Issue 7, pages 1843–1849, July 2011
How to Cite
Lee, S. Y., Usui, S., Zafar, A.-b., Oveson, B. C., Jo, Y.-J., Lu, L., Masoudi, S. and Campochiaro, P. A. (2011), N-acetylcysteine promotes long-term survival of cones in a model of retinitis pigmentosa. J. Cell. Physiol., 226: 1843–1849. doi: 10.1002/jcp.22508
- Issue published online: 19 APR 2011
- Article first published online: 19 APR 2011
- Accepted manuscript online: 10 NOV 2010 12:00AM EST
- Manuscript Accepted: 21 OCT 2010
- Manuscript Received: 28 AUG 2010
- NEI. Grant Number: EY05851
Retinitis pigmentosa (RP) is a major source of blindness caused by a large variety of mutations that lead to the death of rod photoreceptors. After rods die, cones gradually die from progressive oxidative damage. Several types of antioxidant formulations have been shown to reduce cone cell death over a relatively short-time frame, but in order for this strategy to be translated into a new treatment for patients with RP, prolonged effects will be needed. In this study, we determined that orally administered N-acetylcysteine (NAC) reduced cone cell death and preserved cone function by reducing oxidative damage in two models of RP, rd1+/+ and rd10+/+ mice. In rd10+/+ mice, supplementation of drinking water with NAC promoted partial maintenance of cone structure and function for at least 6 months. Topical application of NAC to the cornea also reduced superoxide radicals in the retina and promoted survival and functioning of cones. Since oral and/or topical administration of NAC is feasible for long-term treatment in humans, and NAC has a good safety profile, it is reasonable to consider clinical trials to evaluate the effects of prolonged treatment with NAC in patients with RP. J. Cell. Physiol. 226: 1843–1849, 2011. © 2010 Wiley-Liss, Inc.