Literature DB >> 813921

A case of deficiency of the seventh component of complement in man. Biological properties of a C7-deficient serum and description of a C7-inactivating principle.

B Wellek, W Opferkuch.   

Abstract

The serum of a 13-year-old healthy boy was found to be deficient in whole complement activity. The seventh component of complement could not be detected by functional assays whereas the titres of the other components were found within the normal range. An attempt to detect C7 by immunodiffusion against an antiserum to human C7 also failed. The functions of C1-C6 were normal with respect to opsonizing activity, immune adherence, and the ability to generate chemotactic activity. However, those functions that require the whole complement sequence such as bactericidal and haemolytic activity were found to be absent. Furthermore, the occurrence of a C7-inactivating principle was demonstrated in the C7-deficient serum. This principle inactivated C7 both in the fluid phase and in its cell-bound state. Some physicochemical parameters of the inactivator are described and its possible nature is discussed.

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Year:  1975        PMID: 813921      PMCID: PMC1538101     

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


  26 in total

1.  Further studies on the chemotactic factor of complement and its formation in vivo.

Authors:  P A Ward; C G Cochrane; H J Muller-Eberhard
Journal:  Immunology       Date:  1966-08       Impact factor: 7.397

2.  Hereditary deficiency of the second component of complement (C'2) in man.

Authors:  M R Klemperer; H C Woodworth; F S Rosen; K F Austen
Journal:  J Clin Invest       Date:  1966-06       Impact factor: 14.808

3.  A functional abnormality of the fifth component of complement (C5) from human serum of individuals with a familial opsonic defect.

Authors:  U R Nilsson; M E Miller; S Wyman
Journal:  J Immunol       Date:  1974-03       Impact factor: 5.422

4.  C3 shunt activation in human serum chelated with EGTA.

Authors:  D P Fine; S R Marney; D G Colley; J S Sergent; R M Des Prez
Journal:  J Immunol       Date:  1972-10       Impact factor: 5.422

5.  Hereditary deficiency of the second component of complement (C2) in man: correlation of C2 haemolytic activity with immunochemical measurements of C2 protein.

Authors:  S Ruddy; M R Klemperer; F S Rosen; K F Austen; J Kumate
Journal:  Immunology       Date:  1970-06       Impact factor: 7.397

6.  Hereditary deficiency of the sixth component of complement in man. I. Immunochemical, biologic, and family studies.

Authors:  J P Leddy; M M Frank; T Gaither; J Baum; M R Klemperer
Journal:  J Clin Invest       Date:  1974-02       Impact factor: 14.808

7.  C2 deficiency. Development of lupus erythematosus.

Authors:  N K Day; H Geiger; R McLean; A Michael; R A Good
Journal:  J Clin Invest       Date:  1973-07       Impact factor: 14.808

8.  C1r deficiency: an inborn error associated with cutaneous and renal disease.

Authors:  N K Day; H Geiger; R Stroud; M DeBracco; B Mancaido; D Windhorst; R A Good
Journal:  J Clin Invest       Date:  1972-05       Impact factor: 14.808

9.  Reactive lysis: the complement-mediated lysis of unsensitized cells. II. The characterization of activated reactor as C56 and the participation of C8 and C9.

Authors:  P J Lachmann; R A Thompson
Journal:  J Exp Med       Date:  1970-04-01       Impact factor: 14.307

10.  Reactive lysis: the complement-mediated lysis of unsensitized cells. I. The characterization of the indicator factor and its identification as C7.

Authors:  R A Thompson; P J Lachmann
Journal:  J Exp Med       Date:  1970-04-01       Impact factor: 14.307

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  9 in total

1.  Hereditary deficiency of the fifth component of complement in man. I. Clinical, immunochemical, and family studies.

Authors:  S I Rosenfeld; M E Kelly; J P Leddy
Journal:  J Clin Invest       Date:  1976-06       Impact factor: 14.808

2.  Haemolytic complement in peripheral lymph of normal men.

Authors:  W L Olszewski; A Engeset
Journal:  Clin Exp Immunol       Date:  1978-06       Impact factor: 4.330

3.  Hereditary deficiency of the fifth component of complement in man. II. Biological properties of C5-deficient human serum.

Authors:  S I Rosenfeld; J Baum; R T Steigbigel; J P Leddy
Journal:  J Clin Invest       Date:  1976-06       Impact factor: 14.808

4.  Human deficiency of the eighth component of complement. The requirement of C8 for serum Neisseria gonorrhoeae bactericidal activity.

Authors:  B H Petersen; J A Graham; G F Brooks
Journal:  J Clin Invest       Date:  1976-02       Impact factor: 14.808

5.  Inherited deficiency of the seventh component of complement associated with nephritis. Propensity to formation of C56 and related C7-consuming activity.

Authors:  G R Nemerow; H Gewurz; S G Osofsky; T F Lint
Journal:  J Clin Invest       Date:  1978-06       Impact factor: 14.808

6.  Hereditary C7 deficiency. Diagnosis and HLA studies in a French-Canadian family.

Authors:  J M Delâge; P Bergeron; J Simard; G Lehner-Netsch; E Prochazka
Journal:  J Clin Invest       Date:  1977-11       Impact factor: 14.808

Review 7.  Clinical conditions associated with defective polymorphonuclear leukocyte chemotaxis.

Authors:  P G Quie; K L Cates
Journal:  Am J Pathol       Date:  1977-09       Impact factor: 4.307

8.  Paradoxical reconstitution of complement activity following plasma transfusion of an individual with deficiency of the seventh component of complement.

Authors:  A E Platonov; R Würzner; B Beloborodov; A M Jones; D V Troshansky; I V Vershinina; P J Lachmann; A Orren
Journal:  Immunology       Date:  1994-01       Impact factor: 7.397

9.  Lack of linkage between gene(s) controlling the synthesis of the seventh component of complement and the HLA region on chromosome No. 6 in man.

Authors:  C Rittner; W Opferkuch; B Wellek; H Grosse-Wilde; P Wernet
Journal:  Hum Genet       Date:  1976-10-28       Impact factor: 4.132

  9 in total

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