The complexity of the regulation and signaling of the different GTPases makes it challenging to precisely identify which GTPases are affected by reduced prenylation in MKD, and how this affects different signaling pathways leading to inflammation in different cell types and in vivo. That being said, the finding of a direct link between altered prenylation of the GTPases RhoA and Kras (71, 72) and activation of the pyrin inflammasome in MKD is in full agreement with the early postulation that loss of prenylation is one of the important causes leading to inflammation in MKD (16, 20). Here, RHOA is linked to mevalonic aciduria.