Moreover, an altered flux through the mevalonate pathway is involved in the pathophysiology of the Hyperimmunoglobulin D syndrome (HIDS) and Mevalonic Aciduria (MA), autoinflammatory disorders together known as mevalonate kinase deficiency (MKD) disorders, which are precisely due to a hereditary deficiency of the Mevalonate Kinase (MVK), one of the first enzymes of the mevalonate pathway [6]. The gene discussed is MVK; the disease is mevalonate kinase deficiency.