Biomedical Research

Ribosome biogenesis and mitochondrial function are essential for cellular health, and dysregulation in ribosome production can lead to ribosomopathies. There investigated the effects of deficiencies in ribosomal proteins associated with ribosomopathy disorders, on mitochondrial function using Caenorhabditis elegans and human cells. The authors found that reduced ribosomal protein levels due to a single copy loss of function resulted in developmental delays, altered body sizes, and increased oxidative stress resistance in C. elegans. Conserved changes in translational efficiency and mitochondrial function across species were observed, indicating coordinated regulatory mechanisms. Mitochondrial impairments, including reduced electron transport chain components and compromised energy levels, were identified. In particular, mitochondrial activities were compromised in both C. elegans rps-10 haploinsufficiency mutants and human RPS10-reduced leukemia cells. Finally, unbiased proportionality analysis of RNA and translation efficiency revealed extensive and significant coordinated regulation of ribosomal and mitochondrial genes in human cells. These findings offer insights into cellular resilience mechanisms and potential therapeutic avenues for ribosomopathies.

Author(s): Qiuxia Zhao, Elif Sarinay Cenik
Abstract | PDF

Share this