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

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

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.

Publication types

  • Review

MeSH terms

  • Animals
  • Autoimmune Diseases / immunology*
  • Autoimmune Diseases / pathology
  • Basement Membrane / pathology
  • Basement Membrane / ultrastructure
  • Carcinogenesis / genetics
  • Carcinogenesis / pathology
  • Carcinoma, Squamous Cell / genetics
  • Carcinoma, Squamous Cell / pathology
  • Collagen Type VII / genetics
  • Collagen Type VII / immunology*
  • Collagen Type VII / metabolism
  • Epidermal Cells
  • Epidermis / pathology*
  • Epidermis / ultrastructure
  • Epidermolysis Bullosa Acquisita / immunology*
  • Epidermolysis Bullosa Acquisita / pathology
  • Epidermolysis Bullosa Dystrophica / pathology*
  • Humans
  • Keratinocytes / cytology
  • Keratinocytes / immunology
  • Keratinocytes / pathology
  • Keratinocytes / ultrastructure
  • Microscopy, Electron
  • Skin Neoplasms / genetics
  • Skin Neoplasms / pathology
  • Wound Healing / physiology

Substances

  • Collagen Type VII