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Manganese-driven CoQ deficiency.

Jutta Diessl1, Jens Berndtsson2, Filomena Broeskamp1

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|October 13, 2022
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Summary
This summary is machine-generated.

Manganese overload disrupts cellular energy by inhibiting coenzyme Q (CoQ) production, leading to mitochondrial failure. Restoring CoQ biosynthesis or bypassing the affected enzyme rescues cell viability.

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Area of Science:

  • Biochemistry
  • Cell Biology
  • Toxicology

Background:

  • Manganese overexposure disrupts cellular energy metabolism.
  • The precise molecular mechanisms of manganese toxicity are not fully understood.

Purpose of the Study:

  • To elucidate the molecular mechanism of manganese toxicity.
  • To investigate the impact of manganese on cellular bioenergetics and coenzyme Q biosynthesis.

Main Methods:

  • Investigated the effect of excess manganese on coenzyme Q (CoQ) biosynthesis pathway.
  • Analyzed the interaction of manganese with Coq7, a key enzyme in CoQ production.
  • Assessed mitochondrial bioenergetics and cell viability under manganese overload conditions.
  • Utilized Coq7 overexpression and CoQ analog supplementation to rescue cellular function.

Main Results:

  • Excess manganese selectively inhibits coenzyme Q (CoQ) biosynthesis.
  • Manganese overload causes mismetallation and degradation of the diiron hydroxylase Coq7.
  • Disruption of CoQ biosynthesis leads to impaired mitochondrial electron transport and oxidative phosphorylation.
  • Coq7 overexpression or CoQ analog supplementation restored respiration and cell viability.

Conclusions:

  • Manganese toxicity arises from the disruption of coenzyme Q biosynthesis via Coq7 impairment.
  • This mechanism explains manganese-induced bioenergetic failure conserved across species.
  • Targeting CoQ biosynthesis presents a potential therapeutic strategy for manganese toxicity.