Protects 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. Defects in CPN1 are the cause of carboxypeptidase N deficiency (CPND). 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. Belongs to the peptidase M14 family. Note: This description may include information from UniProtKB.
Protein type: Protease; Secreted, signal peptide; EC 220.127.116.11; Secreted
LTP: The number of records in which this modification site was determined using site-specific methods. SS methods include amino acid sequencing, site-directed mutagenesis, modification site-specific antibodies, specific MS strategies, etc.