Modeling aging and retinal degeneration with mitochondrial DNA mutation burden.

ABSTRACT: Somatic mitochondrial DNA (mtDNA) mutation accumulation has been observed in individuals with retinal degenerative disorders. To study the effects of aging and mtDNA mutation accumulation in the retina, a Polymerase gamma (POLG) deficiency model, the POLG^D257A mutator mice (PolgD257A), was used. POLG is an enzyme responsible for regulating mtDNA replication and repair. Retinas of young and older mice with this mutation were analyzed in vivo and ex vivo to provide new insights into the contribution of age-related mitochondrial dysfunction due to mtDNA damage. Optical coherence tomography (OCT) image analysis revealed a decrease in retinal and photoreceptor thickness starting at 6 months of age in mice with the POLG^D257A mutation compared to wild-type (WT) mice. Electro

SUBMITTER: Sturgis J 

PROVIDER: S-EPMC10705408 | biostudies-literature | 2023 Dec

REPOSITORIES: biostudies-literature