Complete absence of the third component of complement in man.

Ballow, Mark; Shira, J E; Harden, L; Yang, Shan-Yi; Day, Noorbibi K.

doi:10.1172/jci108141

Cited by 110 publications

(35 citation statements)

References 37 publications

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“…The monocytes of nine normal subjects and two C3-deficient patients were studied after obtaining informed consent. The clinical and serological findings in these C3-deficient patients have been reported elsewhere (8)(9)(10). …”

Section: Methodsmentioning

confidence: 66%

“…Established monocyte monolayers were washed with M199 lacking l-lysine, 1-leucine, l-isoleucine, and l-valine (Special M199, SpMl99, Microbiological Associates)3 and then incubated in 0.5 ml of SpM199 supplemented with 10% dialyzed heated fetal calf serum (dHFCS) and 14C-labeled l-lysine (270 mCi/mmol), 1-leucine (270 mCi/mmol), l-isoleucine (270 mCi/mmol), and l-valine (225 mCi/mmol) (New England Nuclear, Boston, Mass.) at a final concentration of 10 ,uCi/ml. After 120 h at 37°C in a humidified 5% CO2:95% air atmosphere, culture medium was removed, immediately centrifuged at 1,700g maximum for 15 min, and stored at -90°C before further analysis.…”

Section: Methodsmentioning

confidence: 99%

“…Deficiency of the third component of complement in man is inherited as an autosomal codominant trait (8)(9)(10)(11).2 Family studies indicated in two kindred that C3 deficiency results from inheritance of a near silent gene (8,12) and metabolic studies of C3 in vivo suggested slight hypercatabolism in homozygous C3 deficients (11, 12) not sufficient to account for the markedly reduced serum C3 concentrations (<1% of normal). In order to study directly the biochemical basis of C3 deficiency, the functional capacities of monocytes from affected patients were compared to monocytes from normal individuals.…”

Section: Introductionmentioning

confidence: 99%

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Biosynthesis of the third component of complement (C3) in vitro by monocytes from both normal and homozygous C3-deficient humans.

Einstein¹,

Hansen²,

Ballow³

et al. 1977

J. Clin. Invest.

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103

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A B S T R A C T Human monocytes synthesized the third component of complement (C3) up to 5 wk in vitro. Evidence for net C3 synthesis was based on (a) incorporation of 14C-labeled amino acids into C3 protein, (b) identity of the allotype of C3 produced in vitro with that of the doner's serum C3, even in the presence of carrier C3 protein of a different allotype; (c) correspondence of electrophoretic mobility, size, and subunit structure of C3 protein produced in vitro with serum C3; (d) inhibition of C3 production with cycloheximide.Monocytes from two unrelated C3-deficient patients were studied under conditions that supported C3 synthesis by normal monocytes. Serum from each of the patients contained <1% of the normal C3 concentration, but their monocytes produced C3 at -25% of the normal rate when studied after 2 wk in vitro. The C3 produced in vitro by monocytes from one of the patients had the molecular weight of normal serum C3 and dissociated appropriately under reducing conditions. Monocytes from C3-deficient patients could not be distinguished from normals on the basis of morphology, rosetting with C3-coated erythrocytes, or rates of C2, and total protein synthesis.

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Section: Methodsmentioning

confidence: 66%

Section: Methodsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Biosynthesis of the third component of complement (C3) in vitro by monocytes from both normal and homozygous C3-deficient humans.

Einstein¹,

Hansen²,

Ballow³

et al. 1977

J. Clin. Invest.

Self Cite

103

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“…Also, CRA was studied in a human serum lacking C3, but containing close to normal concentrations of all other C components of the classical pathway (15). It failed to solubilize immune precipitates even when undiluted, although normal human serum rapidly solubilized the same complexes when diluted one-twentieth.…”

Section: Solubilization Of Ag-abmentioning

confidence: 99%

Studies on the mechanism of solubilization of immune precipitates by serum.

Czop¹,

Nussenzweig²

1976

The Journal of Experimental Medicine

143

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Antigen (Ag)-antibody (Ab) aggregates prepared with several different antigens are solubilized by fresh serum at 37 degrees C (complex-release activity of serum or CRA). The rate of solubilization varies in different systems and is strongly influenced by the affinity of Ab for the Ag in the immune precipitate. With a given Ag-Ab precipitate, the maximum amount of complex that can be solubilized by individual sera is independent of the initial concentration of complexes and cannot be increased by prolonged incubation. CRA occurs in the absence of C2 and C4, but not in the absence of C3 and factor B of the properdin pathway. Addition of C2 to C2-deficient serum or C4 to C4-deficient serum enhances CRA. Solubilization does not involve extensive degradation of the complexed antibody, as might be detected by acrylamide gel electrophoresis of released antibody after reduction and alkylation to separate H and L chains. Immune precipitates can also be solubilized by incubation with monovalent fragments (Fab or Fab') of antibodies against determinants of the Ab molecules in the immune precipitate. In contrast, F(ab')2 fragments decrease the solubility of the immune precipitates. In view of these findings, we propose that CRA is mediated by the binding of functionally monovalent C fragments (C3 and C4) onto Ab molecules in the precipitates.

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“…Hereditary defects of complement components reported thus far mostly involved the classical pathway in human, including Clr (Pickering et al, 1971), Cls (Pondman et al, 1968), C4 (Hauptmann et al, 1974), C2 (Klemperer et al, 1966), C3 (Ballow et al, 1975), C5 (Rosenfeld et al, 1976), C6 (Leddy et al, 1974), C7 (Boyer et al, 1975;Gelgge et aI,, 1977;Nemerow et aL, 1978), and C8 (Petersen et al, 1976), and two inactivators of the complement system, C1 inhibitor (Donaldson and Evans, 1963), and C3b inactivator (Abramson et al, 1971;Thompson and Lachmann, 1977). Few cases of ninth component (C9), the terminal component of the complement sequence, have been reported.…”

Section: Discussionmentioning

confidence: 99%