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Research Article Free access | 10.1172/JCI106013

Inherited deficiency of the third component of human complement (C′3)

Chester A. Alper, Richard P. Propp, Martin R. Klemperer, Fred S. Rosen, and Lillian Watson

Blood Grouping Laboratory and the Department of Medicine, Children's Hospital Medical Center, Boston, Massachusetts 02115

Department of Pediatrics, Harvard Medical School, Boston, Massachusetts 02115

Department of Medicine, Harvard Medical School, Boston, Massachusetts 02115

Find articles by Alper, C. in: PubMed | Google Scholar

Blood Grouping Laboratory and the Department of Medicine, Children's Hospital Medical Center, Boston, Massachusetts 02115

Department of Pediatrics, Harvard Medical School, Boston, Massachusetts 02115

Department of Medicine, Harvard Medical School, Boston, Massachusetts 02115

Find articles by Propp, R. in: PubMed | Google Scholar

Blood Grouping Laboratory and the Department of Medicine, Children's Hospital Medical Center, Boston, Massachusetts 02115

Department of Pediatrics, Harvard Medical School, Boston, Massachusetts 02115

Department of Medicine, Harvard Medical School, Boston, Massachusetts 02115

Find articles by Klemperer, M. in: PubMed | Google Scholar

Blood Grouping Laboratory and the Department of Medicine, Children's Hospital Medical Center, Boston, Massachusetts 02115

Department of Pediatrics, Harvard Medical School, Boston, Massachusetts 02115

Department of Medicine, Harvard Medical School, Boston, Massachusetts 02115

Find articles by Rosen, F. in: PubMed | Google Scholar

Blood Grouping Laboratory and the Department of Medicine, Children's Hospital Medical Center, Boston, Massachusetts 02115

Department of Pediatrics, Harvard Medical School, Boston, Massachusetts 02115

Department of Medicine, Harvard Medical School, Boston, Massachusetts 02115

Find articles by Watson, L. in: PubMed | Google Scholar

Published March 1, 1969 - More info

Published in Volume 48, Issue 3 on March 1, 1969
J Clin Invest. 1969;48(3):553–557. https://doi.org/10.1172/JCI106013.
© 1969 The American Society for Clinical Investigation
Published March 1, 1969 - Version history
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Abstract

A kindred has been investigated in which seven individuals were found to have half-normal serum concentrations of the third component of complement (C′3). This partial deficiency was transmitted as an autosomal dominant trait. Affected individuals were entirely healthy. Hemolytic complement titers were slightly reduced but immune adherence titers and reagent titrations of the classical complement components were normal.

Examination for C′3 allotypes revealed that all affected individuals had patterns resembling those of homozygotes. Analysis of the inheritance of C′3 structural genes disclosed that the most likely mechanism for partial C′3 deficiency in this family was nonexpression of one allele.

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