Document Detail

C1 inhibitor: evidence for decreased hepatic synthesis in hereditary angioneurotic edema.
MedLine Citation:
PMID:  4935379     Owner:  NLM     Status:  MEDLINE    
Although the Cl inhibitor was detected in 5 to 10 percent of normal hepatic parenchymal cells by means of the immunofluorescent technique, none was seen in liver biopsies from two individuals with hereditary angioneurotic edema having low concentrations of Cl inhibitor in the serum. In contrast, the percentages of cells which reacted with fluorescent antiserums to C4 and transferrin were normal. These data suggest that in most subjects with hereditary angioneurotic edema, there is decreased synthesis of the C1 inhibitor but normal synthesis of C4, and that the disease results from this biosynthetic error.
A M Johnson; C A Alper; F S Rosen; J M Craig
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Science (New York, N.Y.)     Volume:  173     ISSN:  0036-8075     ISO Abbreviation:  Science     Publication Date:  1971 Aug 
Date Detail:
Created Date:  1971-10-14     Completed Date:  1971-10-14     Revised Date:  2011-12-13    
Medline Journal Info:
Nlm Unique ID:  0404511     Medline TA:  Science     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  553-4     Citation Subset:  IM    
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MeSH Terms
Angioedema / blood,  genetics,  immunology,  metabolism*
Complement Inactivator Proteins*
Fluorescent Antibody Technique
Liver / analysis,  metabolism*
Middle Aged
Protein Biosynthesis
Reg. No./Substance:
0/Complement Inactivator Proteins

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

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