Plasma levels of C1- inhibitor complexes and cleaved C1- inhibitor in patients with hereditary angioneurotic edema
Articolo
Data di Pubblicazione:
1990
Citazione:
Plasma levels of C1- inhibitor complexes and cleaved C1- inhibitor in patients with hereditary angioneurotic edema / M. Cugno, J. Nuijens, E. Hack, A. Eerenberg, D. Frangi, A. Agostoni, M. Cicardi. - In: THE JOURNAL OF CLINICAL INVESTIGATION. - ISSN 0021-9738. - 85:4(1990 Apr), pp. 1215-1220. [10.1172/JCI114555]
Abstract:
C1- inhibitor (C1(-)-Inh) catabolism in plasma of patients with hereditary angioneurotic edema (HANE) was assessed by measuring the complexes formed by C1(-)-Inh with its target proteases (C1-s, Factor XIIa, and kallikrein) and a modified (cleaved) inactive form of C1(-)-Inh (iC1(-)-Inh). This study was performed in plasma from 18 healthy subjects and 30 patients with HANE in remission: 20 with low antigen concentration (type I) and 10 (from 5 different kindreds) with dysfunctional protein (type II). Both type-I and type-II patients had increased C1(-)-C1(-)-Inh complexes (P less than 0.0001), which in type I inversely correlated with the levels of C1(-)-Inh (P less than 0.001). iC1(-)-Inh was normal in all type-I patients and in type-II patients from three families with increased C1(-)-Inh antigen, whereas iC1(-)-Inh was higher than 20 times the normal values in patients from the remaining two families with C1(-)-Inh antigen in the normal range. None of the subjects had an increase of either Factor XIIa-C1(-)-Inh or kallikrein-C1(-)-Inh complexes. This study shows that the hypercatabolism of C1(-)-Inh in HANE patients at least in part occurs via the formation of complexes with C1- and that genetically determined differences in catabolism of dysfunctional C1(-)-Inh proteins are present in type-II patients
Tipologia IRIS:
01 - Articolo su periodico
Keywords:
C1̄ inhibitor catabolism; C1̄ inhibitor deficiency; Complement; Contact system; Dysfunctional C1̄ inhibitor
Elenco autori:
M. Cugno, J. Nuijens, E. Hack, A. Eerenberg, D. Frangi, A. Agostoni, M. Cicardi
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