Polydatin and Nicotinamide Prevent Iron Accumulation and Lipid Peroxidation in Cellular Models of Mitochondrial Diseases.

ABSTRACT: Ferroptosis, an iron-dependent form of non-apoptotic cell death, is regulated by a complex network involving lipid metabolism, iron homeostasis, and the oxidative-reductive system, with iron accumulation and lipid peroxidation as key drivers. Mitochondrial dysfunction and ROS overproduction often underlie the pathogenesis of mitochondrial diseases, for which treatment options are limited, emphasizing the need for novel therapies. In this study, we investigated whether polydatin and nicotinamide could reverse ferroptosis-related pathological features in cellular models derived from patients with pathogenic GFM1 variants. Mutant fibroblasts showed increased iron and lipofuscin accumulation, altered expression of iron metabolism-related proteins, elevated lipid peroxidation, and height

SUBMITTER: Cilleros-Holgado P 

PROVIDER: S-EPMC11851670 | biostudies-literature | 2025 Feb

REPOSITORIES: biostudies-literature