ADAMTS2
ADAMTS2 is the prototypical procollagen N-proteinase of the ADAMTS family. Its defining substrate is fibrillar procollagen: the enzyme removes the N-terminal propeptides of type I, type II and type III procollagen, an essential step in the maturation of collagen fibrils. Loss-of-function mutations cause dermatosparaxis in cattle and the dermatosparactic form of Ehlers-Danlos syndrome in humans, characterized by extremely fragile, sagging skin. Expression is concentrated in tissues with active collagen deposition such as skin, bone and tendon, making ADAMTS2 a reference enzyme for collagen biosynthesis and connective tissue disease studies. Procollagen processing occurs after secretion, and the enzyme is activated by furin-mediated removal of its prodomain within the secretory pathway.
