Document Detail


Genetically determined heterogeneity of the C1 esterase inhibitor in patients with hereditary angioneurotic edema.
MedLine Citation:
PMID:  4107267     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Normal human serum contains 18 +/-5 mg/100 ml of C1 esterase inhibitor (alpha-2 neuraminoglycoprotein) as estimated by immunochemical means. Of 118 patients with hereditary angioneurotic edema, the sera of 80, from 42 kindred, contained a mean concentration of 3.15 mg/100 ml or 17.5% of normal. The mean serum concentration in 35 patients in 7 other kindred was 20 mg/100 ml or 111% of normal, and 3 patients in another kindred contained over 80 mg/100 ml or greater than 400% of normal. The nonfunctional inhibitors in patients' sera of these eight kindred were identical with normal C1 esterase inhibitor by Ouchterlony analysis, but they were different from normal and from each other with respect to their electrophoretic mobility, their capacity to bind C1 esterase, and their ability to inhibit esterolysis of N-acetyl-tyrosine-ethylester.
Authors:
F S Rosen; C A Alper; J Pensky; M R Klemperer; V H Donaldson
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  The Journal of clinical investigation     Volume:  50     ISSN:  0021-9738     ISO Abbreviation:  J. Clin. Invest.     Publication Date:  1971 Oct 
Date Detail:
Created Date:  1972-01-11     Completed Date:  1972-01-11     Revised Date:  2009-11-18    
Medline Journal Info:
Nlm Unique ID:  7802877     Medline TA:  J Clin Invest     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  2143-9     Citation Subset:  AIM; IM    
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MeSH Terms
Descriptor/Qualifier:
Acetic Acids
Alkanes
Alpha-Globulins / analysis*
Angioedema / enzymology*,  genetics
Animals
Autoradiography
Chromatography, Gel
Complement System Proteins*
Electrophoresis
Esterases / antagonists & inhibitors*
Esters
Glycoproteins / blood*
Goats
Humans
Immunodiffusion
Iodine Isotopes
Metabolism, Inborn Errors / genetics
Neuraminic Acids / blood*
Protein Binding
Radioimmunoassay
Tyrosine
Chemical
Reg. No./Substance:
0/Acetic Acids; 0/Alkanes; 0/Alpha-Globulins; 0/Esters; 0/Glycoproteins; 0/Iodine Isotopes; 0/Neuraminic Acids; 55520-40-6/Tyrosine; 9007-36-7/Complement System Proteins; EC 3.1.-/Esterases
Comments/Corrections

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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