HumanCRB1-Associated Retinal Degeneration: Comparison with therd8 Crb1-Mutant Mouse Model
Investigative Ophthalmology & Visual Science2011Vol. 52(9), pp. 6898–6898
Citations Over TimeTop 10% of 2011 papers
Tomás S. Alemán, Artur V. Cideciyan, Geoffrey K. Aguirre, Wei Huang, Cristina L. Mullins, Alejandro J. Román, Alexander Sumaroka, Melani B. Olivares, Frank F. Tsai, Sharon Schwartz, Luk H. Vandenberghe, Maria P. Limberis, Edwin M. Stone, Peter Bell, James M. Wilson, Samuel G. Jacobson
Abstract
CRB1 mutations lead to early-onset severe loss of vision with thickened, disorganized, nonseeing retina. Impaired peripheral vision can persist in late disease stages. Rd8 mice also have a disorganized retina, but there is sufficient photoreceptor integrity to produce largely normal retinal function. Differences between human and mouse diseases will complicate proof-of-concept studies intended to advance treatment initiatives.
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