Mitochondrial disease, mitophagy, and cellular distress in methylmalonic acidemia.

ABSTRACT: Mitochondria-the intracellular powerhouse in which nutrients are converted into energy in the form of ATP or heat-are highly dynamic, double-membraned organelles that harness a plethora of cellular functions that sustain energy metabolism and homeostasis. Exciting new discoveries now indicate that the maintenance of this ever changing and functionally pleiotropic organelle is particularly relevant in terminally differentiated cells that are highly dependent on aerobic metabolism. Given the central role in maintaining metabolic and physiological homeostasis, dysregulation of the mitochondrial network might therefore confer a potentially devastating vulnerability to high-energy requiring cell types, contributing to a broad variety of hereditary and acquired diseases. In this Review, we highl

SUBMITTER: Luciani A 

PROVIDER: S-EPMC8558192 | biostudies-literature | 2021 Nov

REPOSITORIES: biostudies-literature