Department of Dermatology, Hokkaido University Graduate School of Medicine, Sapporo, Japan.
Division of Dermatology, Niigata University Graduate School of Medical and Dental Sciences, Niigata, Japan.
J Dermatol. 2018 May;45(5):515-521. doi: 10.1111/1346-8138.14222. Epub 2018 Jan 20.
Type VII collagen (COL7), a major component of anchoring fibrils in the epidermal basement membrane zone, has been characterized as a defective protein in dystrophic epidermolysis bullosa and as an autoantigen in epidermolysis bullosa acquisita. Although COL7 is produced and secreted by both epidermal keratinocytes and dermal fibroblasts, the role of COL7 with regard to the epidermis is rarely discussed. This review focuses on COL7 physiology and pathology as it pertains to epidermal keratinocytes. We summarize the current knowledge of COL7 production and trafficking, its involvement in keratinocyte dynamics, and epidermal carcinogenesis in COL7 deficiency and propose possible solutions to unsolved issues in this field.
VII 型胶原（COL7）是表皮基底膜带锚定纤维的主要成分，已被确定为营养不良性大疱性表皮松解症的缺陷蛋白和获得性大疱性表皮松解症的自身抗原。虽然 COL7 由表皮角质形成细胞和真皮成纤维细胞产生和分泌，但关于 COL7 与表皮的关系很少被讨论。这篇综述主要关注 COL7 的生理学和病理学，以及其与表皮角质形成细胞的关系。我们总结了 COL7 产生和运输的最新知识，以及其在角质形成细胞动力学和 COL7 缺乏症中的表皮癌变中的作用，并提出了该领域未解决问题的可能解决方案。
