Hintner H, Breathnach S M, Dahlbäck K, Dahlbäck B, Fritsch P
Universitätsklinik für Dermatologie und Venerologie Innsbruck.
Hautarzt. 1991 Jan;42(1):16-22.
Vitronectin (S-protein of complement, serum spreading factor) is a multifunctional glycoprotein present in human plasma and in the elastic tissue of various organs. It belongs to the group of adhesion proteins and is of importance in the terminal stages of both the coagulation and complement system and in fibrinolysis. In human skin it is localized on dermal elastic fibers and on pathologically altered elastic material (solar elastosis, pseudoxanthoma elasticum) as well as on keratin filament material such as keratin bodies in lichen planus or amyloid deposits in localized cutaneous amyloid. It is also found in the abnormally thickened cutaneous blood vessels in erythropoietic protoporphyria and porphyria cutanea tarda. Late-stage inhibition of the complement cascade in bullous disorders in which activation of the complement system is of pathogenetic significance may be an additional important function of vitronectin in skin diseases.
玻连蛋白(补体S蛋白、血清扩散因子)是一种多功能糖蛋白,存在于人体血浆及各器官的弹性组织中。它属于黏附蛋白组,在凝血系统和补体系统的终末阶段以及纤维蛋白溶解过程中具有重要作用。在人类皮肤中,它定位于真皮弹性纤维、病理改变的弹性物质(日光性弹力纤维变性、弹性假黄瘤)以及角质形成细丝物质,如扁平苔藓中的角质小体或局限性皮肤淀粉样变中的淀粉样沉积物。在红细胞生成性原卟啉症和迟发性皮肤卟啉症中异常增厚的皮肤血管中也能发现它。在补体系统激活具有致病意义的大疱性疾病中,对补体级联反应的晚期抑制可能是玻连蛋白在皮肤疾病中的另一重要功能。