OPA1 Modulates Mitochondrial Ca²⁺ Uptake Through ER-Mitochondria Coupling.

ABSTRACT: Autosomal Dominant Optic Atrophy (ADOA), a disease that causes blindness and other neurological disorders, is linked to OPA1 mutations. OPA1, dependent on its GTPase and GED domains, governs inner mitochondrial membrane (IMM) fusion and cristae organization, which are central to oxidative metabolism. Mitochondrial dynamics and IMM organization have also been implicated in Ca²⁺ homeostasis and signaling but the specific involvements of OPA1 in Ca²⁺ dynamics remain to be established. Here we studied the possible outcomes of OPA1 and its ADOA-linked mutations in Ca²⁺ homeostasis using rescue and overexpression strategies in Opa1-deficient and wild-type murine embryonic fibroblasts (MEFs), respectively and in human ADOA-derived fibroblasts.

SUBMITTER: Cartes-Saavedra B 

PROVIDER: S-EPMC8762365 | biostudies-literature | 2021

REPOSITORIES: biostudies-literature