Figure 3.

Pathogenic mechanisms in MKD. The low residual MK activity in MKD cells is sensitive to increased temperatures, which causes an instant further lowering of the activity and consequently a decreased flux in isoprenoid biosynthesis, which affects the generation of GGPP. The lack of GGPP affects protein geranylgeranylation resulting in ectopic activation of small GTPase, among which GTPases involved in the regulation of the innate immune response. This may cause uncontrolled activation and/or dampening of an inflammatory response.