Mechanisms of impaired mitochondrial homeostasis and NAD⁺ metabolism in a model of mitochondrial heart disease exhibiting redox active iron accumulation.

ABSTRACT: Due to the high redox activity of the mitochondrion, this organelle can suffer oxidative stress. To manage energy demands while minimizing redox stress, mitochondrial homeostasis is maintained by the dynamic processes of mitochondrial biogenesis, mitochondrial network dynamics (fusion/fission), and mitochondrial clearance by mitophagy. Friedreich's ataxia (FA) is a mitochondrial disease resulting in a fatal hypertrophic cardiomyopathy due to the deficiency of the mitochondrial protein, frataxin. Our previous studies identified defective mitochondrial iron metabolism and oxidative stress potentiating cardiac pathology in FA. However, how these factors alter mitochondrial homeostasis remains uncharacterized in FA cardiomyopathy. This investigation examined the muscle creatine kinase conditio

SUBMITTER: Chiang S 

PROVIDER: S-EPMC8379503 | biostudies-literature | 2021 Oct

REPOSITORIES: biostudies-literature