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. 2021 May 12;9:669308. doi: 10.3389/fchem.2021.669308

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Copyright © 2021 Lin, Ren, Gao, Dai and Yu.

This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

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The Biosynthesis and Metabolism of Itaconate. Itaconate is produced by the decarboxylation of cis-aconitate encoded by aconitate decarboxylase 1. Itaconate inhibits SDH and accumulates Succinate. Pyruvate dehydrogenase complex catalyzes pyruvate into citrate precursor—acetyl-CoA.Itaconate is metabolized into itaconyl-coenzyme A. Itaconyl-coenzyme A inactivates mitochondrial CoA B12 thus inhibits methylmalonyl-CoA mutase and methylmalonyl-CoA conversion.