HOMEPRODUCTSCOMPANYCONTACTFAQResearchDictionaryPharmaSign Up FREE or Login

Inherited deficiency of the ninth component of complement in man.

Abstract
A 76-year-old man was found to have low (33% normal) serum complement (C) hemolytic activity, although C3 and C4 protein levels were normal. Further evaluation of his serum and plasma indicated that all C components were present in normal or elevated amounts except for C9, which was undetectable by both antigenic and functional assays. Addition of purified human C9 led to full restoration of the hemolytic activity. Family studies demonstrated that the deficiency was inherited as an autosomal codominant trait and was not linked with alleles at the HLA-A or HLA-B loci. The patient had no history of recurrent or unusual infections and no evidence of autoimmune disease. The availability of serum totally lacking in C9 permitted an investigation of the lytic capacity of the C5b-8 segment of the C attack mechanism, which was pursued in kinetic studies on the hemolysis of erythrocyte intermediates. These studies indicated that hemolysis occurred approximately 100 times slower in patient than in normal serum, using either EA or EAC1-7 intermediates as target cells. Serum bactericidal activity also was slower in patient serum, occurring at a rate about 1/35 that observed in normal serum. These studies provide direct independent evidence that cytolysis of erythrocytes and bacteria can be mediated by C5b-8, and allow a quantitative estimation of the increment in the rates of these reactions provided by normal serum levels of C9. The presence of readily detectable though slow hemolytic activity of C9-deficient serum may account for the difficulty in identifying individuals with this defect.
AuthorsT F Lint, H J Zeitz, H Gewurz
JournalJournal of immunology (Baltimore, Md. : 1950) (J Immunol) Vol. 125 Issue 5 Pg. 2252-7 (Nov 1980) ISSN: 0022-1767 [Print] United States
PMID7430628 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't, Research Support, U.S. Gov't, P.H.S.)
Chemical References
  • Complement C9
  • HLA Antigens
Topics
  • Aged
  • Blood Bactericidal Activity
  • Chromosome Mapping
  • Complement C9 (deficiency, genetics)
  • HLA Antigens
  • Hemolysis
  • Humans
  • Kinetics
  • Male

Join CureHunter, for free Research Interface BASIC access!

Take advantage of free CureHunter research engine access to explore the best drug and treatment options for any disease. Find out why thousands of doctors, pharma researchers and patient activists around the world use CureHunter every day.
Realize the full power of the drug-disease research graph!


Choose Username:
Email:
Password:
Verify Password:
Enter Code Shown: