RPGR-Associated Retinal Degeneration in Human X-Linked RP and a Murine Model
Investigative Ophthalmology & Visual Science2012Vol. 53(9), pp. 5594–5594
Citations Over TimeTop 13% of 2012 papers
Wei Huang, Alan F. Wright, Alejandro J. Román, Artur V. Cideciyan, Forbes Manson, Dina Y. Gewaily, Sharon Schwartz, Sam Sadigh, Maria P. Limberis, Peter Bell, James M. Wilson, Anand Swaroop, Samuel G. Jacobson
Abstract
RPGR mutations lead to progressive loss of rod and cone vision, but show different patterns of residual photoreceptor disease expression. Knowledge of the patterns should guide treatment strategies. Rpgr-cko mice had onset of degeneration at relatively young ages and progressive photoreceptor disease. The natural history in this model will permit preclinical proof-of-concept studies to be designed and such studies should advance progress toward human therapy.
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