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Mitochondria are eukaryotic cellular organelles that are known to produce energy through a process called oxidative phosphorylation. Besides their primary function, mitochondria are involved in various cellular processes, including cell growth, differentiation, signaling, metabolism, and senescence. Age-related changes cause a decline in mitochondrial quality and integrity due to increased mitochondrial mutations and oxidative damage. Thus, aging can severely impact mitochondrial functions,...
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Translation is the process of synthesizing proteins from the genetic information carried by messenger RNA (mRNA). Following transcription, it constitutes the final step in the expression of genes. This process is carried out by ribosomes, complexes of protein and specialized RNA molecules. Ribosomes, transfer RNA (tRNA), and other proteins produce a chain of amino acids—the polypeptide—as the end product of translation.
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Mitochondrial precursors are translocated to the internal subcompartments via independent mechanisms involving distinct protein machineries called translocases.
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Mitochondria are double-membrane organelles of the eukaryotes involved in cellular metabolism, signaling, ATP synthesis, and programmed cell death.  Each of these processes requires specific proteins and enzymes that must be correctly sorted to the right mitochondrial subcompartment for the proper functioning of the organelle.
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Mitochondrial precursors are partially unfolded or loosely folded polypeptide chains. Newly synthesized precursors are inhibited from spontaneously folding into their native conformation by the cytosolic chaperones, heat shock proteins 70 (Hsp70), and mitochondrial import stimulation factors (MSFs). Precursors bound to MSFs are guided to the TOM70-TOM37 receptors, while precursors bound to Hsp70  chaperones are targetted to TOM20-TOM22 receptor complexes.
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Among all the organelles in an animal cell, only mitochondria have their own independent genomes. Animal mitochondrial DNA is a double-stranded, closed-circular molecule with around 20,000 base pairs. Mitochondrial DNA is unique in that one of its two strands, the heavy, or H, -strand is guanine rich, whereas the complementary strand is cytosine rich and called the light, or L, -strand. Compared to nuclear DNA, mitochondrial DNA has a very low percentage of non-coding regions and is marked by...
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Mitochondrial tRNA processing defects reprogram mitochondrial and cellular homeostasis.

Gao Zhu1, Yunfan He2, Xincheng Li2

  • 1Center for Mitochondrial Biomedicine and Department of Otolaryngology-Head and Neck Surgery, The Fourth Affiliated Hospital, Zhejiang University School of Medicine, Yiwu, Zhejiang, China; Institute of Genetics, Zhejiang University International School of Medicine, Hangzhou, Zhejiang, China; Center for Genetic Medicine, Zhejiang University International Institute of Medicine, Yiwu, Zhejiang, China.

The Journal of Biological Chemistry
|June 5, 2025
PubMed
Summary

A mitochondrial tRNA mutation causing deafness disrupts mitochondrial function and quality control. This activates stress responses and mitophagy, ultimately degrading damaged mitochondria to maintain cell balance.

Keywords:
aberrant RNA processingautophagy processdeafnessmitochondrial RNAmitochondrial and cellulamitochondrial dynamicsmitochondrial unfolded protein responsemitophagymutationoxidative phosphorylation

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Area of Science:

  • Cell Biology
  • Genetics
  • Mitochondrial Biology

Background:

  • Mitochondrial tRNA processing defects are linked to clinical conditions like deafness.
  • The m.7516delA mutation specifically impairs RNA precursor processing and mitochondrial translation.

Purpose of the Study:

  • To investigate how the m.7516delA mutation affects organellar and cellular integrity.
  • To elucidate the molecular mechanisms underlying mitochondrial dysfunction caused by this mutation.

Main Methods:

  • Analysis of oxidative phosphorylation (OXPHOS) complex assembly and activity.
  • Assessment of mitochondrial dynamics and morphology.
  • Evaluation of mitophagy pathways (ubiquitin-dependent and independent).
  • Investigation of the integrated stress response (ISR) pathway activation.

Main Results:

  • The mutation altered OXPHOS complexes, impaired mitochondrial dynamics, and increased mitochondrial fission.
  • Both ubiquitin-dependent and independent mitophagy pathways were upregulated, promoting the degradation of damaged mitochondria.
  • The integrated stress response (ISR) pathway was activated, involving key proteins like GCN2, eIF2α, CHOP, ATF4, and ATF5.
  • Activation of ISR and PINK1/Parkin mitophagy pathways enhanced autophagy and apoptotic signaling.

Conclusions:

  • The m.7516delA mutation triggers complex cellular responses to mitigate damage.
  • Aberrant RNA processing leads to mitochondrial dysfunction, altered organelle dynamics, and activation of protective cellular pathways.
  • Findings offer insights into cellular integrity maintenance under stress from genetic defects.