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J Dermatol. 2018 May;45(5):515-521. doi: 10.1111/1346-8138.14222. Epub 2018 Jan 20.

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

Author information

1
Department of Dermatology, Hokkaido University Graduate School of Medicine, Sapporo, Japan.
2
Division of Dermatology, Niigata University Graduate School of Medical and Dental Sciences, Niigata, Japan.

Abstract

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.

KEYWORDS:

COL7A1; epidermolysis bullosa; keratinocytes; squamous cell carcinoma; type VII collagen

PMID:
29352483
DOI:
10.1111/1346-8138.14222
[Indexed for MEDLINE]

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