Journal article

Human Mitoribosome Biogenesis and Its Emerging Links to Disease

Maria Isabel G Lopez Sanchez, Annika Kruger, Dmitrii I Shiriaev, Yong Liu, Joanna Rorbach

INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES | MDPI | Published : 2021

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, high..

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