Quantitative analysis of mitochondrial morphologies in human induced pluripotent stem cells for Leigh syndrome.

ABSTRACT: Mitochondria are dynamic organelles with wide range of morphologies contributing to regulating different signaling pathways and several cellular functions. Leigh syndrome (LS) is a classic pediatric mitochondrial disorder characterized by complex and variable clinical pathologies, and primarily affects the nervous system during early development. It is important to understand the differences between mitochondrial morphologies in healthy and diseased states so that focused therapies can target the disease during its early stages. In this study, we performed a comprehensive analysis of mitochondrial dynamics in five patient-derived human induced pluripotent stem cells (hiPSCs) containing different mutations associated with LS. Our results suggest that subtle alterations in mitochondrial morp

SUBMITTER: Meshrkey F 

PROVIDER: S-EPMC10332439 | biostudies-literature | 2021 Oct

REPOSITORIES: biostudies-literature