Cortical visual function in the rd12 mouse model of Leber Congenital Amarousis (LCA) after gene replacement therapy to restore retinal function.
Vision Res
; 46(22): 3926-34, 2006 Oct.
Article
em En
| MEDLINE
| ID: mdl-16814838
ABSTRACT
One eye of rd12 mice received a sub-retinal injection of a vector carrying normal human RPE65 cDNA at post-natal day 18, and at 6- and 13-months of age. Electroretinograms (ERGs) and visual-evoked potentials (VEPs) were recorded to luminance, and to spatially and temporally modulated stimuli to assess the consequences of delayed treatment on visual pathway function. Early treatment resulted in better overall retinal rescue and better rescue of cone-mediated function. VEPs to low temporal frequency luminance modulation were well preserved at all but the oldest treatment age and corresponded to predictions based on the amount of retinal rescue. In contrast, VEPs to high frequency spatially and temporally modulated stimuli were impaired even at the earliest age. These results provide further support that early treatment in human LCA will have the most hope for optimal visual performance.
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Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Retina
/
Córtex Visual
/
Terapia Genética
/
Cegueira
Tipo de estudo:
Prognostic_studies
Limite:
Animals
Idioma:
En
Revista:
Vision Res
Ano de publicação:
2006
Tipo de documento:
Article
País de afiliação:
Estados Unidos