The branched-chain amino acid-related isoleucic acid: recent research advances.

Isoleucic acid (ILA) was identified in human patients with maple syrup urine disease (MSUD) half a century ago. MSUD patients, who are defective in the catabolism of branched-chain amino acids (BCAAs), that is, isoleucine, leucine, and valine, have urine with a unique maple syrup odour related to the accumulation of BCAA breakdown products, largely 2-keto acid derivatives and their reduced 2-hydroxy acids including ILA. A decade ago, ILA was identified in Arabidopsis thaliana. Subsequent studies in other plant species indicated that ILA is a ubiquitously present compound. Since its identification in plants, several efforts have been made to understand the biological significance and metabolic pathway of ILA. ILA plays a positive role in plant signalling for defence responses against bacterial pathogens by increasing the abundance of salicylic acid aglycone through competitive inhibition of SA deactivation by glucosylation. Here, we review recent progress in the characterization of ILA biosynthesis and function in plants and discuss current knowledge gaps and future directions in ILA research.

© 2025 The Author(s). Plant Biology published by John Wiley & Sons Ltd on behalf of German Society for Plant Sciences, Royal Botanical Society of the Netherlands.