Definition: An extracellular endopeptidase which excises a block of peptides at the amino terminal, nonhelical region of the procollagen molecule with the formation of collagen. Absence or deficiency of the enzyme causes accumulation of procollagen which results in the inherited connective tissue disorder--dermatosparaxis. EC 3.4.24.14.
Other names Procollagen Peptidase; Procollagen N Proteinase; Procollagen N Endopeptidase; Procollagen N-Proteinase
