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Rapid Isolation of the Mitoribosome from HEK Cells
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Elongational stalling activates mitoribosome-associated quality control.

Nirupa Desai1, Hanting Yang1, Viswanathan Chandrasekaran1

  • 1MRC Laboratory of Molecular Biology, Cambridge CB2 0QH, UK.

Science (New York, N.Y.)
|November 27, 2020
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Summary

Researchers discovered a quality control pathway in human mitochondrial ribosomes (mitoribosomes) that rescues stalled protein synthesis. Key proteins eject nascent chains and tRNA, ensuring proper function of oxidative phosphorylation complexes.

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Area of Science:

  • Cell Biology
  • Molecular Biology
  • Biochemistry

Background:

  • The human mitochondrial ribosome (mitoribosome) is crucial for synthesizing 13 essential oxidative phosphorylation (OXPHOS) subunits.
  • Mitoribosome function is vital for cellular energy production.

Purpose of the Study:

  • To discover and characterize a quality control pathway for stalled mitoribosomes.
  • To elucidate the structural mechanisms of ribosome rescue.

Main Methods:

  • Cryo-electron microscopy (cryo-EM) at 3.1- to 4.4-angstrom resolution.
  • Biochemical analysis of protein-RNA and protein-protein interactions.

Main Results:

  • Structures of mitoribosomal large subunits trapped during ribosome rescue were determined.
  • Identified C12orf65 (mtRF-R) and C6orf203 (MTRES1) as key factors in ejecting nascent chains and peptidyl-tRNA.
  • Observed recruitment of mitoribosome biogenesis factors to quality control intermediates.
  • Provided structures of elongating mitoribosomes with elongation factors and Oxa1L.

Conclusions:

  • A novel mitoribosome-associated quality control pathway rescues stalled translation during elongation.
  • mtRF-R and MTRES1 play critical roles in mitoribosome rescue.
  • Mitoribosome biogenesis factors have additional roles in quality control.