Biosynthetic Defect in Monocytes from Human Beings with Genetic Deficiency of the Second Component of Complement

Einstein, L P; Alper, Chester A.; Bloch, Kurt J.; Herrin, John T.; Rosen, Fred S.; David, John R.; Colten, Harvey R.

doi:10.1056/nejm197505292922207

Cited by 55 publications

(12 citation statements)

References 11 publications

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“…The ability of monocytes from C3-deficient patients to produce C3 in vitro stands in contrast to studies of monocytes from C2-deficient humans (24) and macrophages from C4-deficient guinea pigs (25) in which specific and total biosynthetic defects for C2 and C4, respectively, persisted in vitro. In each case, the relevant complement component (C2 or C4) was functionally and immunochemically absent from serum, as well as from monocyte macrophage cultures.…”

Section: Discussioncontrasting

confidence: 73%

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.

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: Discussioncontrasting

confidence: 73%

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.

103

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show abstract

“…Further, antibodies to ENA (1 : 256) were noted in only 1 previous C2D-SLE patient (12) and were of anti-Sm specificity. Discoid lupus was reported in 2 additional C2D individuals ( 5 ) , and a process resembling SLE but more characteristic of Schonlein-Henoch purpura was observed in a third C2D individual (45). Thus, the clinicopathologic findings in Vi T are in distinct contrast with those previously described in C2D-SLE patients and stand out most for their extreme severity, particularly with regard to his renal and cerebral involvement.…”

Section: Discussionmentioning

confidence: 92%

Homozygous C2 deficiency with fulminant lupus erythematosus. severe nephritis via the alternative complement pathway

Gewurz

Lint

Roberts

et al. 1978

Arthritis & Rheumatism

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Absence of C2 was identified in an 18-year-old white male with progressive and rapidly fatal lupus nephritis. Genetic studies of the patient and 8 family members linked this deficiency with the HLA haplotype A10/B18, for which the patient was homozygous. High titers (1 : 1600) of anti-RNP antibodies, as well as antibodies to double-stranded DNA, were present. Serum levels of properdin factor B were persistently decreased, whereas levels of Clq and C3 intermittently were low. Biopsies of skin and kidney showed typical SLE histopathology with deposition of immunoglobulins and early-and late-acting C components; properdin was deposited in the kidney. Despite the inability of the patient's serum to mediate lysis through the classic C pathway, C-dependent lysis through the alternative pathway was readily achieved USing either unsensitized rabbit cells in gels or mixtures of guinea pig cells and zymosan as the indicator system. These studies thus reveal a new association of fatal lupus nephritis with homozygous C2 deficiency and show From the

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“…as described previously (15). Homozygous C2 deficiency was determined by previously published methods (4,24,(26)(27)(28).…”

Section: Methodsmentioning

confidence: 99%

Complement-human histocompatibility antigen haplotypes in C2 deficiency.

Awdeh¹,

Raum²,

Glass³

et al. 1981

J. Clin. Invest.

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Biosynthetic Defect in Monocytes from Human Beings with Genetic Deficiency of the Second Component of Complement

Cited by 55 publications

References 11 publications

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

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

Homozygous C2 deficiency with fulminant lupus erythematosus. severe nephritis via the alternative complement pathway

Complement-human histocompatibility antigen haplotypes in C2 deficiency.

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