AAV-CRB2 protects against vision loss in an inducible CRB1 retinitis pigmentosa mouse model.

ABSTRACT: Loss of Crumbs homolog 1 (CRB1) or CRB2 proteins in Müller cells or photoreceptors in the mouse retina results in a CRB dose-dependent retinal phenotype. In this study, we present a novel Müller cell-specific Crb1 ^KO Crb2 ^LowMGC retinitis pigmentosa mouse model (complete loss of CRB1 and reduced levels of CRB2 specifically in Müller cells). The Crb double mutant mice showed deficits in electroretinography, optokinetic head tracking, and retinal morphology. Exposure of retinas to low levels of dl-α-aminoadipate acid induced gliosis and retinal disorganization in Crb1 ^KO Crb2 ^LowMGC retinas but not in wild-type or Crb1-deficient retinas. Crb1 ^KO Crb2 ^LowMGC mice showed a

SUBMITTER: Buck TM 

PROVIDER: S-EPMC7848734 | biostudies-literature | 2021 Mar

REPOSITORIES: biostudies-literature