Impairment of the transient pupillary light reflex in Rpe65(-/-) mice and humans with leber congenital amaurosis.

@article{Alemn2004ImpairmentOT,
  title={Impairment of the transient pupillary light reflex in Rpe65(-/-) mice and humans with leber congenital amaurosis.},
  author={Tom{\'a}s S. Alem{\'a}n and Samuel G. Jacobson and John D Chico and Michele L Scott and Andy Y. Cheung and Elizabeth A.M. Windsor and Masatoshi Furushima and T Michael Redmond and Jean Bennett and Krzysztof Palczewski and Artur V. Cideciyan},
  journal={Investigative ophthalmology & visual science},
  year={2004},
  volume={45 4},
  pages={1259-71}
}
PURPOSE To determine the impairment of the transient pupillary light reflex (TPLR) due to severe retinal dysfunction and degeneration in a murine model of Leber congenital amaurosis (LCA) and in patients with the disease. METHODS Direct TPLR was elicited in anesthetized, dark-adapted Rpe65(-/-) and control mice with full-field light stimuli (0.1 second duration) of increasing intensities (-6.6 to +2.3 log scot-cd. m(-2)). 9-cis-Retinal was administered orally to a subset of Rpe65(-/-) mice… CONTINUE READING