Human Mitoribosome Biogenesis and Its Emerging Links to Disease.

ABSTRACT: Mammalian mitochondrial ribosomes (mitoribosomes) synthesize a small subset of proteins, which are essential components of the oxidative phosphorylation machinery. Therefore, their function is of fundamental importance to cellular metabolism. The assembly of mitoribosomes is a complex process that progresses through numerous maturation and protein-binding events coordinated by the actions of several assembly factors. Dysregulation of mitoribosome production is increasingly recognized as a contributor to metabolic and neurodegenerative diseases. In recent years, mutations in multiple components of the mitoribosome assembly machinery have been associated with a range of human pathologies, highlighting their importance to cell function and health. Here, we provide a review of our current unde

SUBMITTER: Lopez Sanchez MIG 

PROVIDER: S-EPMC8067846 | biostudies-literature | 2021 Apr

REPOSITORIES: biostudies-literature