β Chain deficiency in three patients with dysfunctional C8 molecules

Francesco Tedesco, Maria A. Villa, Peter Densen, Girolamo Sirchia

Research output: Contribution to journalArticle

Abstract

Structural and functional studies were performed on a dysfunctional C8 molecule present in the serum of two siblings and an unrelated individual. The C8 in these three sera exhibited a pattern of partial immunologic identity with C8 in normal serum but was devoid of functional activity. The C8 was immunoprecipitated from the three sera and from a control serum with an antihuman C8 antiserum and analyzed by SDS-PAGE using highly purified human C8 as a reference. A selective absence of a band of 62,000 mol. wt was observed in the immunoprecipitates from the sera containing dysfunctional C8. Experiments performed with the purified α-γ and γ subunits showed that the hemolytic activity of the C8 deficient sera could be reconstituted by the addition of the β chain but not the α-γ dimer. Binding of the dysfunctional C8 to C567 was excluded by the following observations: (1) EAC 1-7 treated with the C8 deficient sera and then washed could not be lysed after the addition of the β subunit and C9; and (2) the abnormal molecules did not interfere with the consumption of normal C8 by the soluble complex SC5b-7.

Original languageEnglish
Pages (from-to)47-51
Number of pages5
JournalMolecular Immunology
Volume20
Issue number1
DOIs
Publication statusPublished - 1983

ASJC Scopus subject areas

  • Molecular Biology
  • Immunology

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