Dermatosparaxis EDS is due to abnormalities in ADAMTS2 [26], which encodes for an N-proteinase involved in the ablation of N-propeptides whose cleavage is essential for complete maturation of collagen I. Hydroxylation of lysine residues of collagens I and II depends on lysyl hydroxylase 1, encoded by PLOD1, which mutated causes kyphoscoliotic EDS [27]. The gene discussed is ADAMTS2; the disease is Ehlers-Danlos syndrome.