Lack of protein prenylation promotes NLRP3 inflammasome assembly in human monocytes

OP Skinner, J Jurczyluk, PJ Baker… - Journal of Allergy and …, 2019 - jacionline.org
To the Editor: Mevalonate kinase deficiency (MKD; also known as hyper-IgD syndrome) is a
rare autoinflammatory disorder caused by recessive hypomorphic mutations in the MVK
gene. 1 Impaired activity of mevalonate kinase, a crucial component of the mevalonate-
cholesterol biosynthesis pathway, leads to decreased production of isoprenoid lipids,
defective posttranslational protein prenylation, and cytosolic accumulation of unprenylated
Rab proteins and other small GTPases in cells from patients with MKD. 2 It has been …