角蛋白缺陷疾病转基因动物模型的研究进展

Advance of Transgenic Model in Keratin Defect Disease

角蛋白是哺乳动物角质形成细胞的主要结构蛋白,其基因的正确表达是细胞稳定和功能正常的基础。角蛋白的基因突变可导致一系列的遗传性疾病。近年来,转基因动物模型的建立在疾病的发病机制、基因间的相互关系方面带给了我们全新的视角。本文就角蛋白相关疾病动物模型的研究现状做一综述。

Keratin is the major structural protein in mammalian keratinocyte. The correct expression of this gene affects the stable and the basic function of the cell. The mutation of these genes can lead to many kinds of genetic disease. In recent years, the transgenic animal model gives us a new perspective in pathogenesis of the disease and the correlation of gene. The current review summarized the advance of the transgenic model make for keratin defect disease.