Cytosolic Ribosomal Protein Haploinsufficiency affects Mitochondrial Morphology and Respiration.

ABSTRACT: The interplay between ribosomal protein composition and mitochondrial function is essential for sustaining energy homeostasis. Precise stoichiometric production of ribosomal proteins is crucial to maximize protein synthesis efficiency while reducing the energy costs to the cell. However, the impact of this balance on mitochondrial ATP generation, morphology and function remains unclear. Particularly, the loss of a single copy ribosomal protein gene is observed in Mendelian disorders like Diamond Blackfan Anemia and is common in somatic tumors, yet the implications of this imbalance on mitochondrial function and energy dynamics are still unclear. In this study, we investigated the impact of haploinsufficiency for four ribosomal protein genes implicated in ribosomopathy disorders (rps-10,

SUBMITTER: Surya A 

PROVIDER: S-EPMC11042305 | biostudies-literature | 2024 May

REPOSITORIES: biostudies-literature