Department of Dermatology, Medical Center - University of Freiburg, Faculty of Medicine, University of Freiburg, Germany.
Eur J Dermatol. 2017 Jun 1;27(S1):8-11. doi: 10.1684/ejd.2017.3039.
Focal adhesions are large multiprotein cell-matrix adhesion complexes, which regulate multiple cellular functions, such as adhesion and migration. Their biological significance in skin is underscored by two genetic disorders, the Kindler syndrome and the interstitial lung disease, nephrotic syndrome and epidermolysis bullosa, in which mutations affect focal adhesion proteins, kindlin-1 and the integrin α3 subunit, respectively. Here we provide an overview of what we learned from the study of the molecular mechanisms of these diseases. Emphasis is put on the point of view of the clinician dermatologist.
黏着斑是一种大型的细胞基质黏附复合物,它可以调节多种细胞功能,如黏附和迁移。在皮肤中,有两种遗传疾病凸显了黏着斑的生物学意义,即 Kindler 综合征和间质性肺病、肾病综合征和大疱性表皮松解症,这些疾病的突变分别影响黏着斑蛋白、连接蛋白-1 和整合素α3 亚基。本文概述了我们从这些疾病的分子机制研究中学到的知识。重点介绍了皮肤科医生的观点。