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rdf:type |
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lifeskim:mentions |
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pubmed:issue |
4
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pubmed:dateCreated |
1990-5-9
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pubmed:abstractText |
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.
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pubmed:commentsCorrections |
http://linkedlifedata.com/resource/pubmed/commentcorrection/2318974-1275365,
http://linkedlifedata.com/resource/pubmed/commentcorrection/2318974-14046003,
http://linkedlifedata.com/resource/pubmed/commentcorrection/2318974-14223932,
http://linkedlifedata.com/resource/pubmed/commentcorrection/2318974-14257837,
http://linkedlifedata.com/resource/pubmed/commentcorrection/2318974-14461960,
http://linkedlifedata.com/resource/pubmed/commentcorrection/2318974-2440799,
http://linkedlifedata.com/resource/pubmed/commentcorrection/2318974-2445044,
http://linkedlifedata.com/resource/pubmed/commentcorrection/2318974-2563376,
http://linkedlifedata.com/resource/pubmed/commentcorrection/2318974-2668333,
http://linkedlifedata.com/resource/pubmed/commentcorrection/2318974-2841334,
http://linkedlifedata.com/resource/pubmed/commentcorrection/2318974-3146615,
http://linkedlifedata.com/resource/pubmed/commentcorrection/2318974-3178731,
http://linkedlifedata.com/resource/pubmed/commentcorrection/2318974-3264190,
http://linkedlifedata.com/resource/pubmed/commentcorrection/2318974-3289579,
http://linkedlifedata.com/resource/pubmed/commentcorrection/2318974-3388291,
http://linkedlifedata.com/resource/pubmed/commentcorrection/2318974-3493816,
http://linkedlifedata.com/resource/pubmed/commentcorrection/2318974-3734104,
http://linkedlifedata.com/resource/pubmed/commentcorrection/2318974-376558,
http://linkedlifedata.com/resource/pubmed/commentcorrection/2318974-3818946,
http://linkedlifedata.com/resource/pubmed/commentcorrection/2318974-3875374,
http://linkedlifedata.com/resource/pubmed/commentcorrection/2318974-3923103,
http://linkedlifedata.com/resource/pubmed/commentcorrection/2318974-3965500,
http://linkedlifedata.com/resource/pubmed/commentcorrection/2318974-4107267,
http://linkedlifedata.com/resource/pubmed/commentcorrection/2318974-433606,
http://linkedlifedata.com/resource/pubmed/commentcorrection/2318974-4956917,
http://linkedlifedata.com/resource/pubmed/commentcorrection/2318974-6168693,
http://linkedlifedata.com/resource/pubmed/commentcorrection/2318974-6343376,
http://linkedlifedata.com/resource/pubmed/commentcorrection/2318974-6587741,
http://linkedlifedata.com/resource/pubmed/commentcorrection/2318974-6601240,
http://linkedlifedata.com/resource/pubmed/commentcorrection/2318974-6833491,
http://linkedlifedata.com/resource/pubmed/commentcorrection/2318974-6902743,
http://linkedlifedata.com/resource/pubmed/commentcorrection/2318974-6976242,
http://linkedlifedata.com/resource/pubmed/commentcorrection/2318974-7172497
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pubmed:language |
eng
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pubmed:journal |
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pubmed:citationSubset |
AIM
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pubmed:chemical |
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pubmed:status |
MEDLINE
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pubmed:month |
Apr
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pubmed:issn |
0021-9738
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pubmed:author |
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pubmed:issnType |
Print
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pubmed:volume |
85
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
1215-20
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pubmed:dateRevised |
2009-11-18
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pubmed:meshHeading |
pubmed-meshheading:2318974-Adolescent,
pubmed-meshheading:2318974-Adult,
pubmed-meshheading:2318974-Aged,
pubmed-meshheading:2318974-Angioedema,
pubmed-meshheading:2318974-Complement C1 Inactivator Proteins,
pubmed-meshheading:2318974-Electrophoresis, Polyacrylamide Gel,
pubmed-meshheading:2318974-Female,
pubmed-meshheading:2318974-Humans,
pubmed-meshheading:2318974-Male,
pubmed-meshheading:2318974-Middle Aged,
pubmed-meshheading:2318974-Molecular Weight,
pubmed-meshheading:2318974-Mutation
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pubmed:year |
1990
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pubmed:articleTitle |
Plasma levels of C1- inhibitor complexes and cleaved C1- inhibitor in patients with hereditary angioneurotic edema.
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pubmed:affiliation |
Cattedra di Clinica Medica Università di Milano, Ospedale S. Paolo, Italy.
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pubmed:publicationType |
Journal Article,
Research Support, Non-U.S. Gov't
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