Epidermal aspects of type VII collagen: Implications for dystrophic epidermolysis bullosa and epidermolysis bullosa acquisita

被引:18
作者
Watanabe, Mika [1 ]
Natsuga, Ken [1 ]
Shinkuma, Satoru [2 ]
Shimizu, Hiroshi [1 ]
机构
[1] Hokkaido Univ, Dept Dermatol, Grad Sch Med, North 15 West 7, Sapporo, Hokkaido 0608638, Japan
[2] Niigata Univ, Div Dermatol, Grad Sch Med & Dent Sci, Niigata, Japan
关键词
COL7A1; epidermolysis bullosa; keratinocytes; squamous cell carcinoma; type VII collagen; SQUAMOUS-CELL CARCINOMA; BONE-MARROW-TRANSPLANTATION; PLURIPOTENT STEM-CELLS; BASEMENT-MEMBRANE; REVERTANT MOSAICISM; GENE-EXPRESSION; STRUCTURAL ORGANIZATION; PROCOLLAGEN-VII; JUNCTION ZONE; MESSENGER-RNA;
D O I
10.1111/1346-8138.14222
中图分类号
R75 [皮肤病学与性病学];
学科分类号
100206 ;
摘要
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.
引用
收藏
页码:515 / 521
页数:7
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