BGLAP and body skin hyperlaxity due to vitamin K-dependent coagulation factor deficiency: Interestingly, Vanakker et al. confirmed an accumulation of uncarboxylated MGP and OC in patients with the PXE-like syndrome; similarly, an in vitro model for the mutation leading to the Keutel syndrome-like phenotype in P7 showed an abolishment of MGP carboxylation [28,36].