Document Detail

Genetic defect in secretion of complement C5 in mice.
MedLine Citation:
PMID:  492335     Owner:  NLM     Status:  MEDLINE    
A genetic deficiency of the fifth (C5) component of complement1-3, a serum glycoprotein of molecular weight (MW) 220,000 (ref. 4), has been found in 39% of inbred strains of mice3. Sera of deficient mice lack detectable C5 activity and protein2,3. In addition deficient mice produce antibody to mouse C5 when injected with sera from C5 sufficient (normal) strains. Levy et al.5 showed that somatic cell hybrids between C5 deficient (B10.D2/old line) macrophages and either C5 sufficient (B10.D2/new line) mouse kidney or chicken erythroblasts secreted haemolytically active mouse C5 in vitro. Several possible molecular mechanisms to account for the findings were considered, but insufficient direct data were available to choose among them. We recently reported that mouse (CD.1 strain) peritoneal cells in culture synthesise and secrete a single chain precursor, pro-C5 (MW approximately 210,000), of the two-chain (alpha chain, 125,000 and beta chain 83,000 MW) C5 protein6. Radiolabelled precursor C5 was contained within the cells and was secreted into the tissue culture media. Using similar methods, we now find that C5 deficiency in each of five different mouse strains (AKR, SWR, DBA/2J8 A/HeJ and B10.D2/old line) is due to a failure in secretion of C5 protein and not to a failure in biosynthesis of pro-C5.
Y M Ooi; H R Colten
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Nature     Volume:  282     ISSN:  0028-0836     ISO Abbreviation:  Nature     Publication Date:  1979 Nov 
Date Detail:
Created Date:  1979-12-29     Completed Date:  1979-12-29     Revised Date:  2005-11-17    
Medline Journal Info:
Nlm Unique ID:  0410462     Medline TA:  Nature     Country:  ENGLAND    
Other Details:
Languages:  eng     Pagination:  207-8     Citation Subset:  IM    
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MeSH Terms
Ascitic Fluid / cytology
Cells, Cultured
Complement C5 / deficiency,  genetics,  metabolism*
Extracellular Space / metabolism
Glycoproteins / genetics
Guinea Pigs
Macrophages / metabolism
Protein Precursors / metabolism
Reg. No./Substance:
0/Complement C5; 0/Glycoproteins; 0/Protein Precursors

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