Exacerbated response to oxidative stress in the Retinitis Pigmentosa Cerkl^KD/KO mouse model triggers retinal degeneration pathways upon acute light stress.

ABSTRACT: The retina is particularly vulnerable to genetic and environmental alterations that generate oxidative stress and cause cellular damage in photoreceptors and other retinal neurons, eventually leading to cell death. CERKL (CERamide Kinase-Like) mutations cause Retinitis Pigmentosa and Cone-Rod Dystrophy in humans, two disorders characterized by photoreceptor degeneration and progressive vision loss. CERKL is a resilience gene against oxidative stress, and its overexpression protects cells from oxidative stress-induced apoptosis. Besides, CERKL contributes to stress granule-formation and regulates mitochondrial dynamics in the retina. Using the Cerkl^KD/KO albino mouse model, which recapitulates the human disease, we aimed to study the impact of Cerkl knockdown on stress response a

SUBMITTER: Garcia-Arroyo R 

PROVIDER: S-EPMC10491808 | biostudies-literature | 2023 Oct

REPOSITORIES: biostudies-literature