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- W2079970798 abstract "Using in vitro complement immunofluorescent staining methods, serum samples from 5 active cases of bullous pemphigoid,with pemphigoid antibody titers of 320 or greater, were tested for their ability to fix the regulatory protein β 1H globulin in addition to C4 and C3. All 5 samples yielded positive C3, C4 and β 1H staining reactions in a linear fashion along the basement membrane zone. Heat inactivation or treatment of the complement source (fresh normal human serum) with EDTA, Mg 2 -EGTA abolished all 3 staining reactions. Substitution of C2-deficient serum as the source of complement inhibited both C3 and β1H staining but had no effect on C4 staining. Use of serum devoid of β 1H (R β 1H) minimally enhanced C3 staining while no β 1H staining was observed. The addition of β 1H to R β 1H restored positive β 1H staining. Skin biopsies of perilesional skin from 6 patients with bullous pemphigoid demonstrated heavy in vivo deposition of β 1H in addition to C3. These studies suggest that pemphigoid antibodies will fix the regulatory protein β 1H in addition to other complement components, a phenomenon which requires activation of the classical complement pathway and generation of the C3b amplification convertase." @default.
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- W2079970798 date "1980-12-01" @default.
- W2079970798 modified "2023-09-26" @default.
- W2079970798 title "The Complement System in Bullous Pemphigoid: VII. Fixation of the Regulatory Protein β1H Globulin by Pemphigoid Antibody" @default.
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- W2079970798 doi "https://doi.org/10.1111/1523-1747.ep12524224" @default.
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