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CO2-sensitive tRNA modification associated with human mitochondrial disease.

Huan Lin1, Kenjyo Miyauchi1, Tai Harada1

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Mitochondrial N⁶-threonylcarbamoyladenosine (t⁶A) formation is regulated by CO₂/bicarbonate levels, impacting mitochondrial translation and potentially causing disease. This discovery challenges the view of static tRNA modifications.

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Area of Science:

  • Molecular Biology
  • Biochemistry
  • Genetics

Background:

  • Transfer RNA (tRNA) modifications were traditionally considered stable and static.
  • N⁶-threonylcarbamoyladenosine (t⁶A) is a crucial modification at position 37 of five mitochondrial (mt-)tRNA species.

Purpose of the Study:

  • To identify the enzymes responsible for t⁶A37 formation in mitochondria.
  • To investigate the regulatory mechanisms and physiological relevance of t⁶A37.
  • To explore the link between t⁶A37 deficiency and pathological conditions.

Main Methods:

  • Enzyme assays using L-threonine, ATP, and CO₂/bicarbonate.
  • Generation and analysis of OSGEPL1-knockout cells.
  • Isolation and analysis of mt-tRNA from patient-derived cells and cultured human cells.
  • Kinetic measurements of t⁶A37 formation.

Main Results:

  • YRDC and OSGEPL1 were identified as the enzymes responsible for t⁶A37 synthesis.
  • OSGEPL1-knockout cells displayed significant respiratory defects and reduced mitochondrial translation.
  • A low t⁶A37 level was observed in mt-tRNA from a MERRF-like patient, correlating with pathological consequences.
  • CO₂/bicarbonate was identified as a rate-limiting substrate with a high Km value (31 mM).
  • Reduced t⁶A37 frequency was observed in human cells cultured without bicarbonate.

Conclusions:

  • t⁶A37 formation is dynamically regulated by intracellular CO₂/bicarbonate concentration.
  • This regulation implies a mechanism for modulating mitochondrial translation in a codon-specific manner.
  • Dysregulation of t⁶A37 can lead to pathological conditions, highlighting its importance in mitochondrial health.