Nicein (BM-600) in junctional epidermolysis bullosa: polyclonal antibodies provide new clues for pathogenic role.
Nicein (BM-600) in junctional epidermolysis bullosa: polyclonal antibodies provide new clues for pathogenic role.
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交界性大疱性表皮松解症中的 Nicein (BM-600)：多克隆抗体为致病作用提供新线索。
DOI：
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发表时间：
1993
影响因子：
6.5
通讯作者：
J. Ortonne
中科院分区：
文献类型：
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作者：
P. Verrando;O. Schofield;A. Ishida‐Yamamoto;D. Aberdam;O. Partouche;R. Eady;J. Ortonne
我们已经针对新的基底膜成分Nicein(以前的BM-600)的三个亚基中的每一个提出了多克隆抗体，该抗原被单抗GB3识别(Biochem BiPhys Acta 942：45-56,1988)。采用凝胶电泳法对免疫亲和层析法分离纯化的Nicein进行纯化，制备抗体。这些抗体与每个转印的变性Nicein亚基发生反应，并在培养的角质形成细胞和所有GB3抗原所在的正常人类基底膜中识别天然蛋白。针对Nicein的100-kD和150-kD(有时可分解为146-150-kD双重链)亚基的抗体具有相互的免疫交叉反应，表明它们具有相同的表位。在Herlitz型交界性大疱性表皮松解症(H-Jeb)患者的组织和角质形成细胞培养中，GB3无法识别Nicein，问题是这是由于缺乏合成还是由于蛋白质的结构异常。我们在这里报道，针对Nicein 150-kD亚基的多克隆抗体在H-Jeb患者中与其抗原结合(尽管通常不如对照皮肤那么强烈)，而其他两种抗体要么不识别，要么只识别它们各自的抗原亚基。这些数据表明，在H-Jeb组织的基底膜中存在Nicein，但结构发生了变化。此外，在非Herlitz交界型和营养不良型大疱性表皮松解症中，所有三种多克隆抗体都能正常识别它们的抗原。因此，这种抗体应该成为研究Nicein在H-Jeb中表达的潜在有用的分子工具。
We have raised polyclonal antibodies against each of three subunits of the new basement membrane component nicein (formerly BM-600), the antigen recognized by the monoclonal antibody GB3 (Biochem Biophys Acta 942:45-56, 1988). Preparation of such antibodies was achieved from gel electrophoresis purification of nicein isolated by immuno-affinity chromatography. These antibodies were reactive with each transblotted denatured nicein subunit and recognized the native protein both in cultured keratinocytes and in all normal human basement membranes where the GB3 antigen is located. A reciprocal immuno-cross-reactivity was detected with the antibodies directed against the 100-kD and 150-kD (sometimes resolved as a 146-150-kD doublet) subunits of nicein, showing that they share some identical epitopes. In tissues and keratinocyte cultures from patients with the Herlitz form of junctional epidermolysis bullosa (H-JEB), GB3 is unable to recognize nicein, and the question arises whether this is due to an absence of synthesis or a structural abnormality of the protein. We report here that the polyclonal antibody directed against the 150-kD subunit of nicein binds its antigen in H-JEB patients (although usually less intensely than in control skin), whereas the other two antibodies either do not recognize or recognize only weakly their respective antigen subunits. These data suggest that nicein is present but structurally altered in basement membranes from H-JEB tissues. Furthermore, in non-Herlitz junctional and dystrophic types of epidermolysis bullosa, all three polyclonal antibodies recognize their antigens normally. Consequently, such antibodies should serve as potentially useful molecular tools for studying the expression of nicein in H-JEB.
GB3 单克隆抗体用于诊断大疱性交界性表皮松解症：多中心研究的结果。
影响因子：
13.8
作者：
Schofield,OM;Fine,JD;Verrando,P;Heagerty,AH;Ortonne,JP;Eady,RA
通讯作者：
Eady,RA
19-DEJ-1 是半桥粒锚定丝复合物的单克隆抗体，是用于所有主要形式的交界性大疱性表皮松解症的唯一可靠的免疫组织化学探针。
影响因子：
--
作者：
Fine,JD
通讯作者：
Fine,JD