FunctionProtects the body from potent vasoactive and inflammatory peptides containing C-terminal Arg or Lys (such as kinins or anaphylatoxins) which are released into the circulation.
Tissue specificitySynthesized in the liver and secreted in plasma.
Involvement in diseaseDefects in CPN1 are the cause of carboxypeptidase N deficiency (CPND) [MIM:212070]. Patients affected present some combination of angioedema or chronic urticaria, as well as hay fever or astma, and have also slightly depressed serum carboxy peptidase N, suggestive of autosomal recessive inheritance of this disorder.
Sequence similaritiesBelongs to the peptidase M14 family.