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首页> 美国卫生研究院文献>The Journal of Clinical Investigation >Identification of the cutaneous basement membrane zone antigen and isolation of antibody in linear immunoglobulin A bullous dermatosis.
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Identification of the cutaneous basement membrane zone antigen and isolation of antibody in linear immunoglobulin A bullous dermatosis.

机译：线性免疫球蛋白A大疱性皮肤病中皮肤基底膜区抗原的鉴定和抗体的分离。

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Linear IgA bullous dermatosis (LABD) is a rare blistering skin disease characterized by basement membrane zone deposition of IgA. This study identifies a tissue antigen detected by patient serum and then isolates the autoantibody using epidermis and protein bands blotted on nitrocellulose as immunoabsorbents. Sera from 10 patients (9 with cutaneous disease and 1 with cicatrizing conjunctivitis) were evaluated. Indirect immunofluorescence revealed an IgA anti-basement membrane antibody in 6 of 10 sera with monkey esophagus substrate and 9 of 10 sera with human epidermal substrate. Immunoblotting was performed on epidermal and dermal extracts prepared from skin separated at the basement membrane zone with either sodium chloride or EDTA. Saline-separated skin expressed a 97-kD band in dermal extract alone that was recognized by 4 of 10 sera. EDTA-separated skin expressed the 97-kD band in both epidermal (4 of 10 sera) and dermal (6 of 10 sera) extract. Immunoabsorption of positive sera with epidermis purified an IgA antibody that reacted uniquely with the 97-kD band. In addition, IgA antibody bound to nitrocellulose was eluted from the 97-kD band and found to uniquely bind basement membrane zone. It is likely that the 97-kD protein identified by these techniques is responsible for basement membrane binding of IgA in LABD.
机译：线性IgA大疱性皮肤病（LABD）是一种罕见的水疱性皮肤病，其特征在于IgA的基底膜区沉积。这项研究鉴定了由患者血清检测到的组织抗原，然后使用在硝酸纤维素上印迹的表皮和蛋白带作为免疫吸收剂分离自身抗体。评估了10例患者的血清（9例皮肤疾病和1例瘢痕结膜炎）。间接免疫荧光法显示，在10份血清中有6份具有猴食管底物，而9份血清中有9份具有人表皮底物，IgA抗碱膜抗体。对用氯化钠或EDTA从在基底膜区分离的皮肤制备的表皮和真皮提取物进行免疫印迹。盐水分离的皮肤仅在真皮提取物中表达97-kD条带，可被10个血清中的4个识别。 EDTA分离的皮肤在表皮（每10个血清中有4个）和真皮（每10个血清中有6个）提取物中均表达97-kD条带。表皮对阳性血清的免疫吸收纯化了与97-kD条带唯一反应的IgA抗体。另外，从97-kD条带上洗脱出与硝酸纤维素结合的IgA抗体，并发现其与基底膜区唯一结合。通过这些技术鉴定出的97-kD蛋白可能是LABD中IgA的基底膜结合的原因。

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