Meneguzzi G, Marinkovich M P, Aberdam D, Pisani A, Burgeson R, Ortonne J P
Laboratoire de Recherches Dermatologiques, Université de Nice-Sophia Antipolis-06108 Nice, France.
Exp Dermatol. 1992 Dec;1(5):221-9. doi: 10.1111/j.1600-0625.1992.tb00080.x.
Kalinin is an extracellular adhesion molecule specific to epithelial basement membranes (BM) identified as a component of anchoring filaments of hemidesmosomes. This heterotrimeric protein is synthesized by cultured normal human keratinocytes and is involved in cell attachment. In indirect immunofluorescence studies, the epidermal BM of patients with junctional epidermolysis bullosa (JEB) of Herlitz's type were found not to be reactive with the anti-kalinin monoclonal antibodies ka146 and K140 and displayed a decreased immunoreactivity to two anti-kalinin antibodies cross-reacting with K-laminin, an anchoring filament component recently described. The intrinsic defect of JEB keratinocytes in the synthesis of kalinin was further documented by indirect immunofluorescence on in vitro cultures of these cells. In non-Herlitz JEB patients, staining of BM was constantly detected. Impairment of expression of kalinin correlated with the lack of reactivity to the monoclonal antibody GB3, which detects the BM component nicein/BM600. These results clearly demonstrate a defect of kalinin expression in epithelial basement membranes of Herlitz JEB patients and suggest that kalinin may play a role in the pathogenesis of the disease. Further studies are in progress to define possible relationships between kalinin and nicein.
角蛋白是一种上皮基底膜（BM）特有的细胞外黏附分子，被确定为半桥粒锚定丝的一个组成部分。这种异源三聚体蛋白由培养的正常人角质形成细胞合成，并参与细胞黏附。在间接免疫荧光研究中，发现赫利茨型交界性大疱性表皮松解症（JEB）患者的表皮基底膜与抗角蛋白单克隆抗体ka146和K140无反应，并且对两种与K-层粘连蛋白（最近描述的一种锚定丝成分）交叉反应的抗角蛋白抗体的免疫反应性降低。通过对这些细胞的体外培养进行间接免疫荧光，进一步证实了JEB角质形成细胞在角蛋白合成方面的内在缺陷。在非赫利茨型JEB患者中，始终能检测到基底膜的染色。角蛋白表达的受损与对检测基底膜成分尼斯汀/BM600的单克隆抗体GB3缺乏反应性相关。这些结果清楚地证明了赫利茨型JEB患者上皮基底膜中角蛋白表达存在缺陷，并表明角蛋白可能在该疾病的发病机制中起作用。正在进行进一步的研究以确定角蛋白与尼斯汀之间可能的关系。
