Decreased synthesis of serum carboxypeptidase N (SCPN) in familial SCPN deficiency.
Serum carboxypeptidase N (SCPN) is the primary inactivator of the C3a, C4a, and C5a anaphylatoxins as well as an inactivator of bradykinin. Thus SCPN deficiency potentially could result in significant pathophysiologic consequences. Previous studies identified a deficient subject afflicted with frequent episodes of angioedema, and other family members also had SCPN deficiency. To delineate this abnormality further, the fractional catabolic rate (FRC) and enzyme synthesis were determined in three members of the afflicted kindred as well as in five normal persons following the infusion of homogeneous 125I-SCPN. The mean FCR and synthesis rates for SCPN in the normal subjects were 1.3%/hr and 20,793 U/kg/hr, respectively. Reduced synthesis was concluded to be primarily responsible for the low SCPN levels in the afflicted kindred. The high FRC of SCPN discourages attempted maintenance therapy with infusions of enriched SCPN preparations.
D002268:Carboxypeptidases / Q000097:blood*; D006801:Humans; D007457:Iodine Radioisotopes; D007700:Kinetics; D001121:Lysine Carboxypeptidase / Q000096:biosynthesis / Q000097:blood* / Q000172:deficiency; D012016:Reference Values
D007457:Iodine Radioisotopes; D002268:Carboxypeptidases; D001121:Lysine Carboxypeptidase