Simplified schematic overview of the mevalonate pathway, which is distributed at the level of mevalonate kinase in mevalonate kinase deficiency
Bold text in dashed boxes are enzymes. Italicized text in solid-line boxes are drugs/agents that block the enzymatic pathways.
From: Stoffels M, Simon A. Hyper-IgD syndrome or mevalonate kinase deficiency. Curr Opin Rheumatol 2011; 23:419. DOI:
10.1097/BOR.0b013e328349c3b1. Copyright © 2011. Reproduced with permission from Wolters Kluwer Health. Unauthorized reproduction of this material is prohibited.