The experiments presented here utilize a human serum markedly deficient in hemolytic complement activity to show that: (a) The hemolytic deficiency is the result of a selective deficiency in hemolytic C1. (b) The relative absence of hemolytic C1 is due to a profound deficit in C1r function associated with less than normal C1s protein and hemolytic function and normal C1q protein concentration and function. This deficit in C1r in the face of normal C1q suggests that different cell types are responsible for the synthesis of each of these components. (c) Whatever the basis for the deficiency of C1r function, this defect results in an inadequate association of the remaining C1 subcomponents, C1q and C1s, even in the presence of calcium ions, thus suggesting that C1r has an important role in the assembly and/or maintenance of macromolecular C1.
Article|
April 01 1970
DEFICIENCY OF C1r IN HUMAN SERUM : EFFECTS ON THE STRUCTURE AND FUNCTION OF MACROMOLECULAR C1
R. J. Pickering,
R. J. Pickering
From the Pediatric Research Laboratories of the Variety Club Heart Hospital, University of Minnesota, Minneapolis, Minnesota 55455, and the Departments of Medicine, Case Western Reserve University and University of Alabama
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G. B. Naff,
G. B. Naff
From the Pediatric Research Laboratories of the Variety Club Heart Hospital, University of Minnesota, Minneapolis, Minnesota 55455, and the Departments of Medicine, Case Western Reserve University and University of Alabama
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R. M. Stroud,
R. M. Stroud
From the Pediatric Research Laboratories of the Variety Club Heart Hospital, University of Minnesota, Minneapolis, Minnesota 55455, and the Departments of Medicine, Case Western Reserve University and University of Alabama
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R. A. Good,
R. A. Good
From the Pediatric Research Laboratories of the Variety Club Heart Hospital, University of Minnesota, Minneapolis, Minnesota 55455, and the Departments of Medicine, Case Western Reserve University and University of Alabama
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H. Gewurz
H. Gewurz
From the Pediatric Research Laboratories of the Variety Club Heart Hospital, University of Minnesota, Minneapolis, Minnesota 55455, and the Departments of Medicine, Case Western Reserve University and University of Alabama
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R. J. Pickering
From the Pediatric Research Laboratories of the Variety Club Heart Hospital, University of Minnesota, Minneapolis, Minnesota 55455, and the Departments of Medicine, Case Western Reserve University and University of Alabama
G. B. Naff
From the Pediatric Research Laboratories of the Variety Club Heart Hospital, University of Minnesota, Minneapolis, Minnesota 55455, and the Departments of Medicine, Case Western Reserve University and University of Alabama
R. M. Stroud
From the Pediatric Research Laboratories of the Variety Club Heart Hospital, University of Minnesota, Minneapolis, Minnesota 55455, and the Departments of Medicine, Case Western Reserve University and University of Alabama
R. A. Good
From the Pediatric Research Laboratories of the Variety Club Heart Hospital, University of Minnesota, Minneapolis, Minnesota 55455, and the Departments of Medicine, Case Western Reserve University and University of Alabama
H. Gewurz
From the Pediatric Research Laboratories of the Variety Club Heart Hospital, University of Minnesota, Minneapolis, Minnesota 55455, and the Departments of Medicine, Case Western Reserve University and University of Alabama
Received:
November 06 1969
Online ISSN: 1540-9538
Print ISSN: 0022-1007
Copyright © 1970 by The Rockefeller University Press
1970
J Exp Med (1970) 131 (4): 803–815.
Article history
Received:
November 06 1969
Citation
R. J. Pickering, G. B. Naff, R. M. Stroud, R. A. Good, H. Gewurz; DEFICIENCY OF C1r IN HUMAN SERUM : EFFECTS ON THE STRUCTURE AND FUNCTION OF MACROMOLECULAR C1 . J Exp Med 1 April 1970; 131 (4): 803–815. doi: https://doi.org/10.1084/jem.131.4.803
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