CPN1 Antibody – Rabbit

Carboxypeptidase N is a plasma metallo-protease that cleaves basic amino acids from the C terminal of peptides and proteins. The enzyme is important in the regulation of peptides like kinins and anaphylatoxins, and has also been known as kininase-1 and anaphylatoxin inactivator. This enzyme is a tetramer comprised of two identical regulatory subunits and two identical catalytic subunits; this gene encodes the catalytic subunit. Mutations in this gene can be associated with angioedema or chronic urticaria resulting from carboxypeptidase N deficiency. [provided by RefSeq, Jul 2008]

CPN1: 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.

Protein type: EC 3.4.17.3; Secreted, signal peptide; Protease; Secreted

Chromosomal Location of Human Ortholog: 10q24.2

Cellular Component: extracellular space

Molecular Function: zinc ion binding; metallocarboxypeptidase activity

Biological Process: response to glucocorticoid stimulus; proteolysis

Disease: Carboxypeptidase N Deficiency