Antigenic expression of integrin α6β4 in junctional epidermolysis bullosa
Antigenic expression of integrin α6β4 in junctional epidermolysis bullosa
复制标题
整合素α6β4 在交界性大疱性表皮松解症中的抗原表达
作者：
Robert J. Phillips;John D. Aplin;B. D. Lake
整合素α6β4是半粒粒的主要成分，被认为在基底表皮细胞与真皮的粘附中起核心作用。因此，对遗传性起疱性疾病的病因学研究具有相当大的兴趣。我们检测了16例大泡性表皮松解症患者皮肤的免疫组织化学特征，使用了两种针对αβ4整合素β4亚基表位的抗体（G71, 3E1），一种针对α6亚基表位的抗体（GoH3），一种针对nicein的抗体GB3和一种抗VII型胶原抗体LH7.2。10例大疱性结缔组织表皮松解症患者G71免疫反应性均明显降低或无反应。这些患者包括2例与幽门闭锁相关的GB3阳性结性大疱性表皮松解症，以及4例其他亚型。5例营养不良大疱性表皮松解症患者和1例单纯大疱性表皮松解症患者G71免疫反应正常。本研究显示G71对大疱性结缔组织表皮松解症的诊断具有很高的特异性和敏感性。大多数患者与3E1和GoH3的免疫反应正常，与已发表的报告显示与其他β4和α6亚基抗体的免疫反应正常一致。这些数据表明，在大疱性表皮松解症中，整合素α6β4的β4亚基在真皮-表皮交界处发生了修饰。
Integrin α6β4 is a major component of hemidesmosomes, considered to play a central role in the adhesion of basal epidermal cells to the underlying dermis. It is therefore of considerable interest in the study of the aetiology of inherited blistering disorders. We have examined the immunohistochemical characteristics of skin from 16 patients with epidermolysis bullosa using two antibodies directed against epitopes on the β4 subunit of αβ4 integrin (G71, 3E1), one antibody directed against an epitope on the α6 subunit (GoH3), GB3 an antibody for nicein, and LH7.2, an anti‐collagen type VII antibody. All 10 patients with junctional epidermolysis bullosa showed markedly reduced or no immunoreactivity with G71. These patients included two with GB3‐positive junctional epidermolysis bullosa associated with pyloric atresia, and four with other subtypes. By contrast, five patients with dystrophic epidermolysis bullosa and one patient with epidermolysis bullosa simplex showed normal immunoreactivity with G71. In this study, G71 is shown to have a high specificity and sensitivity for the diagnosis of junctional epidermolysis bullosa. Immunoreactivity with 3E1 and GoH3 was normal in most patients, consistent with published reports showing normal immunoreactivity with other β4 and α6 subunit antibodies. The data suggest a modification of the β4 subunit of integrin α6β4 at the dermo‐epidermal junction in junctional epidermolysis bullosa.
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：
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作者：
Fine,JD
通讯作者：
Fine,JD