canine and human visual cortex intact and responsive despite early retinal blindness from rpe65 mutation狗和人类视觉皮层完好无损,响应尽管早期视网膜失明rpe65突变.pdfVIP

PLoS MEDICINE Canine and Human Visual Cortex Intact and Responsive Despite Early Retinal Blindness from RPE65 Mutation 1* ´ ´ 2 3 4 3 Geoffrey K. Aguirre , Andras M. Komaromy , Artur V. Cideciyan , David H. Brainard , Tomas S. Aleman , 3 5 5 1 6 7 Alejandro J. Roman , Brian B. Avants , James C. Gee , Marc Korczykowski , William W. Hauswirth , Gregory M. Acland , 2 3 Gustavo D. Aguirre , Samuel G. Jacobson 1 Department of Neurology, School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, United States of America, 2 Department of Clinical Studies, School of Veterinary Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, United States of America, 3 Department of Ophthalmology, School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, United States of America, 4 Department of Psychology, School of Arts and Sciences, University of Pennsylvania, Philadelphia, Pennsylvania, United States of America, 5 Department of Radiology, School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, United States of America, 6 Department of Ophthalmology, University of Florida, Gainesville, Florida, 7 Baker Institute, College of Veterinary Medicine, Cornell University, Ithaca, New York, United States of America Funding: See section at end of A B S T R A C T manuscript. Competing Interests: See section at end of manuscript. Background Academic Editor: Alvaro Pascual- Leone, Beth Israel Deaconess RPE65 is an essential molecule in the retinoid-visual cycle, and RPE65 gene mutations cause Medica