KENNETH P. MATHEWS, M.D.; PAULINE M. PAN, A.B.; NEVEN J. GARDNER, M.D.; TONY E. HUGLI, Ph.D.
Carboxypeptidase N is a serum metalloenzyme that inactivates C3a, C4a, C5a, bradykinin, kalladin, and fibrinopeptides. Of 172 sera from patients with chronic urticaria or angioedema, one had a remarkably depressed carboxypeptidase N level (21% of normal). Of sera from 103 patients with other diseases, elevated levels were observed in cases of neoplasms, and one abnormally low value was detected in a patient with cirrhosis. The patient with a remarkably low carboxypeptidase N level was a 65-year-old man with an 11-year history of episodic angioedema occurring about 40 times per year. Inactivation of C3a and lysyl-bradykinin by his serum was markedly prolonged. Plasma histamine was elevated during attacks, but serotonin and kinin activity were not. The proband's sister had an equally depressed serum carboxypeptidase N level, and studies of other family members suggested an autosomal recessive inheritance of the enzyme deficiency.
MATHEWS KP, PAN PM, GARDNER NJ, et al. Familial Carboxypeptidase N Deficiency. Ann Intern Med. 1980;93:443–445. doi: https://doi.org/10.7326/0003-4819-93-3-443
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Published: Ann Intern Med. 1980;93(3):443-445.
Gastroenterology/Hepatology, Hematology/Oncology, Liver Disease.
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Print ISSN: 0003-4819 | Online ISSN: 1539-3704
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