Mitochondrial tRNA processing defects reprogram mitochondrial and cellular homeostasis.

ABSTRACT: Mitochondrial tRNA processing defects have been associated with some clinical presentations including deafness. Especially, a deafness-linked m.7516delA mutation impaired the 5' end processing of RNA precursors and mitochondrial translation. In this study, we investigated the mechanism by m.7516delA mutation-induced deficiencies mitigate organellar and cellular integrity. The m.7516delA mutation downregulated the expression of nucleus-encoding subunits and upregulated assembly factors of complex IV and altered the assembly and activities of oxidative phosphorylation (OXPHOS) complexes. The impairment of OXPHOS alleviated mitochondrial quality control processes, including the imbalanced mitochondrial dynamics via increasing fission with abnormal mitochondrial morphology. The m.7516delA muta

SUBMITTER: Zhu G 

PROVIDER: S-EPMC12269583 | biostudies-literature | 2025 Jun

REPOSITORIES: biostudies-literature