Open AccessEarly-Onset Progressive Degeneration of the Area Centralis in RPE65-Deficient Dogs
Author(s) -
Freya M. Mowat,
Kristen J. Gervais,
Laurence M. Occelli,
Matthew J. Annear,
Janice Querubin,
James Bainbridge,
Alexander J. Smith,
Robin R. Ali,
Simon M. PetersenJones
Publication year - 2017
Publication title -
investigative ophthalmology and visual science
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.935
H-Index - 218
eISSN - 1552-5783
pISSN - 0146-0404
DOI - 10.1167/iovs.17-21930
Subject(s) - rpe65 , retinal degeneration , retina , electroretinography , ophthalmology , retinal , photoreceptor cell , macular degeneration , outer nuclear layer , cis trans isomerases , retinal pigment epithelium , degeneration (medical) , medicine , anatomy , biology , pathology , neuroscience , peptidylprolyl isomerase , biochemistry , isomerase , gene
Retinal epithelium-specific protein 65 kDa (RPE65)-deficient dogs are a valuable large animal model species that have been used to refine gene augmentation therapy for Leber congenital amaurosis type-2 (LCA2). Previous studies have suggested that retinal degeneration in the dog model is slower than that observed in humans. However, the area centralis of the dog retina is a cone and rod photoreceptor rich region comparable to the human macula, and the effect of RPE65 deficiency specifically on this retinal region, important for high acuity vision, has not previously been reported.
Accelerating Research
Robert Robinson Avenue,
Oxford Science Park, Oxford
OX4 4GP, United Kingdom
Address
John Eccles HouseRobert Robinson Avenue,
Oxford Science Park, Oxford
OX4 4GP, United Kingdom