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For a long time, itaconic acid was only known as a metabolic product of fungi. Its function was unknown, but for decades it has been used industrially for polymer production. In 2013, it was surprisingly discovered as an important metabolic product in the immune system. This newly described role has sparked a search for potential clinical applications. Researchers at the Helmholtz Centre for Infection Research (HZI) and TWINCORE-Centre for Experimental and Clinical Infection Research, jointly founded by the HZI and the Hanover Medical School, have now solved the crystal structure of the itaconic acid-synthesizing enzyme cis-aconitate decarboxylase (CAD). Their findings provide the basis for an improved understanding of itaconic acid synthesis, the evolutionary origin of this important metabolic step, and the role of itaconic acid in the immune system. In particular, the researchers identified naturally occurring and possibly clinically important variations in the human CAD enzyme.