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Small Molecules as Alternate Substrates for 3-Methylglutaconylation.

Elizabeth A Jennings1, Irina Romenskaia1, Robert O Ryan1

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|October 17, 2025
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Summary

Inborn errors of metabolism can cause toxic buildup of trans-3-methylglutaconyl CoA. This study shows primary amine-containing molecules can react with reactive 3MGC anhydride, preventing protein damage.

Keywords:
3MGC anhydride3‐methylglutaconyl‐CoA hydrataseHMG‐CoA lyasemetabolite acylationprimary amine

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Area of Science:

  • Biochemistry
  • Metabolic Disorders
  • Organic Chemistry

Background:

  • Leucine catabolism produces trans-3-methylglutaconyl CoA (3MGC-CoA).
  • Enzyme deficiencies in HMGCL or AUH (inborn errors of metabolism) elevate 3MGC-CoA levels.
  • Elevated 3MGC-CoA can non-enzymatically form 3MGC anhydride, leading to organic acid waste and protein 3MGCylation.

Purpose of the Study:

  • Investigate the reactivity of 3MGC anhydride with small molecules.
  • Assess the potential of small molecules to mitigate 3MGC anhydride-induced protein modification.

Main Methods:

  • In vitro experiments using an anti-3MGC antibody.
  • Assessing the reduction in 3MGCylated bovine serum albumin (BSA) signal intensity.
  • Testing various small molecules, including amino group-containing compounds.

Main Results:

  • Glycine, glucosamine, ethanolamine, and glutathione reduced 3MGCylated BSA signal intensity in a concentration-dependent manner.
  • These reactive molecules contain primary amine groups.
  • N-acetylglucosamine and choline did not show significant effects.

Conclusions:

  • 3MGC anhydride reacts with primary amine-containing metabolites.
  • This reaction acylates the primary amine groups of these metabolites.
  • Potential therapeutic strategy for managing toxic metabolite buildup in specific IEMs.