Lee C W, Jordon R E
J Invest Dermatol. 1980 Dec;75(6):465-9. doi: 10.1111/1523-1747.ep12524224.
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 beta 1H globulin in addition to C4 and C3. All 5 samples yielded positive C3, C4 and beta 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, Mg2-EGTA abolished all 3 staining reactions. Substitution of C2-deficient serum as the source of complement inhibited both C3 and beta 1H staining but had no effect on C4 staining. Use of serum devoid of beta 1H (R beta 1H) minimally enhanced C3 staining while no beta 1H staining was observed. The addition of beta 1H to R beta 1H restored positive beta 1H staining. Skin biopsies of perilesional skin from 6 patients with bullous pemphigoid demonstrated heavy in vivo deposition of beta 1H in addition to C3. These studies suggest that pemphigoid antibodies will fix the regulatory protein beta 1H in addition to other complement components, a phenomenon which requires activation of the classical complement pathway and generation of the C3b amplification convertase.
采用体外补体免疫荧光染色法，对5例天疱疮抗体效价为320或更高的大疱性类天疱疮活动期患者的血清样本进行检测，以评估其除结合C4和C3外，结合调节蛋白β1H球蛋白的能力。所有5份样本在基底膜区均呈现沿基底膜区呈线性的阳性C3、C4和β1H染色反应。对补体来源（新鲜正常人血清）进行热灭活或用EDTA、Mg2 - EGTA处理可消除所有3种染色反应。用缺乏C2的血清替代补体来源可抑制C3和β1H染色，但对C4染色无影响。使用不含β1H的血清（Rβ1H）可轻微增强C3染色，而未观察到β1H染色。向Rβ1H中添加β1H可恢复阳性β1H染色。对6例大疱性类天疱疮患者的皮损周围皮肤进行活检，结果显示除C3外，β1H在体内有大量沉积。这些研究表明，类天疱疮抗体除结合其他补体成分外，还能结合调节蛋白β1H，这一现象需要经典补体途径的激活和C3b放大转化酶的产生。
