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Research Article Free access | 10.1172/JCI106902
Department of Pathology, University of Minnesota, Minneapolis, Minnesota 55455
Department of Pediatrics, University of Minnesota, Minneapolis, Minnesota 55455
Department of Medicine, University of Alabama, Birmingham, Alabama 35233
Division of Dermatology, University of Chicago, Chicago, Illinois 60601
Find articles by Day, N. in: JCI | PubMed | Google Scholar
Department of Pathology, University of Minnesota, Minneapolis, Minnesota 55455
Department of Pediatrics, University of Minnesota, Minneapolis, Minnesota 55455
Department of Medicine, University of Alabama, Birmingham, Alabama 35233
Division of Dermatology, University of Chicago, Chicago, Illinois 60601
Find articles by Geiger, H. in: JCI | PubMed | Google Scholar
Department of Pathology, University of Minnesota, Minneapolis, Minnesota 55455
Department of Pediatrics, University of Minnesota, Minneapolis, Minnesota 55455
Department of Medicine, University of Alabama, Birmingham, Alabama 35233
Division of Dermatology, University of Chicago, Chicago, Illinois 60601
Find articles by Stroud, R. in: JCI | PubMed | Google Scholar
Department of Pathology, University of Minnesota, Minneapolis, Minnesota 55455
Department of Pediatrics, University of Minnesota, Minneapolis, Minnesota 55455
Department of Medicine, University of Alabama, Birmingham, Alabama 35233
Division of Dermatology, University of Chicago, Chicago, Illinois 60601
Find articles by deBracco, M. in: JCI | PubMed | Google Scholar
Department of Pathology, University of Minnesota, Minneapolis, Minnesota 55455
Department of Pediatrics, University of Minnesota, Minneapolis, Minnesota 55455
Department of Medicine, University of Alabama, Birmingham, Alabama 35233
Division of Dermatology, University of Chicago, Chicago, Illinois 60601
Find articles by Mancado, B. in: JCI | PubMed | Google Scholar
Department of Pathology, University of Minnesota, Minneapolis, Minnesota 55455
Department of Pediatrics, University of Minnesota, Minneapolis, Minnesota 55455
Department of Medicine, University of Alabama, Birmingham, Alabama 35233
Division of Dermatology, University of Chicago, Chicago, Illinois 60601
Find articles by Windhorst, D. in: JCI | PubMed | Google Scholar
Department of Pathology, University of Minnesota, Minneapolis, Minnesota 55455
Department of Pediatrics, University of Minnesota, Minneapolis, Minnesota 55455
Department of Medicine, University of Alabama, Birmingham, Alabama 35233
Division of Dermatology, University of Chicago, Chicago, Illinois 60601
Find articles by Good, R. in: JCI | PubMed | Google Scholar
Published May 1, 1972 - More info
The studies of sera from two siblings with C1r deficiency are described. The brother (18 yr old) has shown clinical manifestations resembling lupus erythematosus for 5 yr, and the sister (24 yr old) has had arthralgia and recurrent episodes of rhinobronchitis since early childhood. Three siblings have died: one brother died at age 12 with symptoms similar to the disease of the male patient studied here, and two other siblings died in infancy, probably from infection. The low hemolytic C1 activity of the patients could be restored by the addition of purified C1r to their sera. Bactericidal activity and immune adherence were found to be impaired. When alternate pathways of the complement system were studied, both sera permitted activation of terminal components with endotoxin and cobra venom factor. These findings support the view that an alternate pathway for activation of the terminal portion of the complement cascade exists which does not utilize the conventional pathway operating through the usual early components.
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