Component deficiencies. 8. The eighth component

Research output: Contribution to journalArticle

Abstract

The study of C8-deficient patients has revealed clinical findings essentially similar to those found in patients with defects of other lateacting complement components being characterized primarily by a high incidence of Neisseria infections. The reason why these patients are not equally susceptible to infections by gram-negative bacteria other than Neisseria is not apparent but may partly be explained by a selection bias due to the more extensive search for genetic deficiencies of the terminal components among patients with Neisseria infections. Furthermore, the finding of serum-resistant strains of Neisseria in some of these patients raises some questions as to the interpretation of the mechanisms involved in the protection against Neisseria infections by the terminal components of the complement sequence. The identification of two groups of subjects with C8 deficiency, the C8α-γ deficient and the C8β deficient, is the most relevant observation regarding the inheritance of this defect and is compatible with the hypothesis that two genentic loci control the synthesis of C8. Information on the site of synthesis of these two subunits is lacking, although the liver appears to be a likely candidate.

Original languageEnglish
Pages (from-to)295-306
Number of pages12
JournalProgress in Allergy
VolumeVol. 39
Publication statusPublished - 1986

ASJC Scopus subject areas

  • Immunology and Allergy

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