Literature DB >> 7094424

C3b inactivator deficiency with immune complex manifestations.

P Solal-Celigny, M Laviolette, J Hebert, P C Atkins, M Sirois, G Brun, G Lehner-Netsch, J M Delâge.   

Abstract

We report a complete C3b inactivator deficiency in a 28-year-old patient referred for bronchiectasis and recurrent pneumonia. In addition to these recurrent infections, previously described with this deficiency, he also had several immune complex manifestations (rheumatoid factor, circulating immune complexes and one episode of serum sickness). The consequences of C3b inactivator deficiency on complement activation, chemotaxis and opsonization, clearance of immune complexes, and on red blood cell sensitization are discussed. The study of the parents showed an autosomal dominant transmission.

Entities:  

Mesh：

Substances：

Year:  1982        PMID： 7094424      PMCID： PMC1536350     

Source DB:  PubMed          Journal:  Clin Exp Immunol        ISSN： 0009-9104            Impact factor:   4.330


  21 in total

1.  Lysis of human erythrocytes due to activation of the alternate complement pathway by nephritic factor (C3NeF).

Authors:  C M Arroyave; E H Vallota; H J Müller-Eberhard
Journal:  J Immunol       Date:  1974-09       Impact factor: 5.422

2.  Activation of the alternate pathway of human complements by rabbit cells.

Authors:  T A Platts-Mills; K Ishizaka
Journal:  J Immunol       Date:  1974-07       Impact factor: 5.422

3.  [Modified technic for the measurement of CI esterase inhibitor].

Authors:  H Brécy; L Hartmann
Journal:  Biomedicine       Date:  1973-08-10

4.  The stoichiometric measurement of the serum inhibition of the first component of complement by the inhibition of immune hemolysis.

Authors:  I Gigli; S Ruddy; K F Austen
Journal:  J Immunol       Date:  1968-06       Impact factor: 5.422

5.  Deficiency of C3 inactivator in man.

Authors:  N Abramson; C A Alper; P J Lachmann; F S Rosen; J H Jandl
Journal:  J Immunol       Date:  1971-07       Impact factor: 5.422

6.  Detection of immune complexes in unheated sera by modified 125I-Clq binding test. Effect of heating on the binding of Clq by immune complexes and application of the test to systemic lupus erythematosus.

Authors:  R H Zubler; G Lange; P H Lambert; P A Miescher
Journal:  J Immunol       Date:  1976-01       Impact factor: 5.422

7.  Restoration by purified C3b inactivator of complement-mediated function in vivo in a patient with C3b inactivator deficiency.

Authors:  J B Ziegler; C A Alper; R S Rosen; P J Lachmann; L Sherington
Journal:  J Clin Invest       Date:  1975-03       Impact factor: 14.808

8.  Inactivator of the third component of complement as an inhibitor in the properdin pathway.

Authors:  C A Alper; F S Rosen; P J Lachmann
Journal:  Proc Natl Acad Sci U S A       Date:  1972-10       Impact factor: 11.205

9.  Studies in vivo and in vitro on an abnormality in the metabolism of C3 in a patient with increased susceptibility to infection.

Authors:  C A Alper; N Abramson; R B Johnston; J H Jandl; F S Rosen
Journal:  J Clin Invest       Date:  1970-11       Impact factor: 14.808

10.  The mechanism of action of the C3b inactivator (conglutinogen-activating factor) on its naturally occurring substrate, the major fragment of the third component of complement (C3b).

Authors:  J D Gitlin; F S Rosen; P J Lachmann
Journal:  J Exp Med       Date:  1975-05-01       Impact factor: 14.307
View more
  9 in total

1.  Recurrent infections in partial complement factor I deficiency: evaluation of three generations of a Brazilian family.

Authors:  A S Grumach; M F Leitão; V G Arruk; M Kirschfink; A Condino-Neto
Journal:  Clin Exp Immunol       Date:  2006-02       Impact factor: 4.330

2.  Complement-activating ability of leucocytes from patients with complement factor I deficiency.

Authors:  H V Marquart; J M Rasmussen; R G Leslie
Journal:  Immunology       Date:  1997-07       Impact factor: 7.397

3.  The molecular basis of hereditary complement factor I deficiency.

Authors:  T J Vyse; B J Morley; I Bartok; E L Theodoridis; K A Davies; A D Webster; M J Walport
Journal:  J Clin Invest       Date:  1996-02-15       Impact factor: 14.808

Review 4.  Complement deficiency and immune complex disease.

Authors:  K A Davies; J A Schifferli; M J Walport
Journal:  Springer Semin Immunopathol       Date:  1994

5.  Functional deficiency of complement factor D in a monozygous twin.

Authors:  H C Kluin-Nelemans; H van Velzen-Blad; H P van Helden; M R Daha
Journal:  Clin Exp Immunol       Date:  1984-12       Impact factor: 4.330

6.  Generation of multiple fluid-phase C3b:plasma protein complexes during complement activation: possible implications in C3 glomerulopathies.

Authors:  Mahalakshmi Ramadass; Berhane Ghebrehiwet; Richard J Smith; Richard R Kew
Journal:  J Immunol       Date:  2013-12-23       Impact factor: 5.422

7.  Three cases of factor I deficiency: the effect of treatment with plasma.

Authors:  J Møller Rasmussen; B Teisner; H H Jepsen; S E Svehag; F Knudsen; H Kirstein; M Buhl
Journal:  Clin Exp Immunol       Date:  1988-10       Impact factor: 4.330

8.  Complete factor I deficiency due to dysfunctional factor I with recurrent aseptic meningo-encephalitis.

Authors:  Filomeen Haerynck; Patrick Stordeur; Johan Vandewalle; Rudy Van Coster; Victoria Bordon; Frans De Baets; Petra Schelstraete; Cédric Javaux; Marie-Rose Bouvry; Véronique Fremeaux-Bacchi; Joke Dehoorne
Journal:  J Clin Immunol       Date:  2013-10-20       Impact factor: 8.317

9.  Complete and partial deficiencies of complement factor D in a Dutch family.

Authors:  P S Hiemstra; E Langeler; B Compier; Y Keepers; P C Leijh; M T van den Barselaar; D Overbosch; M R Daha
Journal:  J Clin Invest       Date:  1989-12       Impact factor: 14.808
  9 in total

北京卡尤迪生物科技股份有限公司 © 2022-2023.