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线状免疫球蛋白A大疱性皮肤病中皮肤基底膜带抗原的鉴定及抗体的分离

Identification of the cutaneous basement membrane zone antigen and isolation of antibody in linear immunoglobulin A bullous dermatosis.

作者信息

Zone J J, Taylor T B, Kadunce D P, Meyer L J

机构信息

Department of Internal Medicine, University of Utah School of Medicine, Salt Lake City 84132.

出版信息

J Clin Invest. 1990 Mar;85(3):812-20. doi: 10.1172/JCI114508.

DOI:10.1172/JCI114508
PMID:2107211
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC296499/
Abstract

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.

摘要

线状IgA大疱性皮肤病(LABD)是一种罕见的水疱性皮肤病,其特征为IgA在基底膜带沉积。本研究鉴定了一种可被患者血清检测到的组织抗原,然后以硝酸纤维素膜上印迹的表皮和蛋白条带作为免疫吸附剂分离自身抗体。评估了10例患者的血清(9例患有皮肤病,1例患有瘢痕性结膜炎)。间接免疫荧光显示,10份血清中有6份在猴食管底物上、9份血清在人表皮底物上出现IgA抗基底膜抗体。对用氯化钠或EDTA在基底膜带分离的皮肤制备的表皮和真皮提取物进行免疫印迹分析。用盐水分离的皮肤仅在真皮提取物中表达一条97-kD条带,10份血清中有4份可识别该条带。用EDTA分离的皮肤在表皮提取物(10份血清中有4份)和真皮提取物(10份血清中有6份)中均表达97-kD条带。用表皮对阳性血清进行免疫吸附纯化出一种IgA抗体,该抗体仅与97-kD条带发生反应。此外,从97-kD条带洗脱与硝酸纤维素膜结合的IgA抗体,发现其仅与基底膜带结合。通过这些技术鉴定的97-kD蛋白可能是LABD中IgA与基底膜结合的原因。

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