Blistering disease: insight from the hemidesmosome and other components of the dermal-epidermal junction
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  • 作者:Iana Turcan ; Marcel F. Jonkman
  • 关键词:Hemidesmosome ; Dermal ; epidermal junction ; Bullous disease ; Epidermolysis bullosa ; Pemphigoid
  • 刊名:Cell and Tissue Research
  • 出版年:2015
  • 出版时间:June 2015
  • 年:2015
  • 卷:360
  • 期:3
  • 页码:545-569
  • 全文大小:9,720 KB
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  • 作者单位:Iana Turcan (1)
    Marcel F. Jonkman (1)

    1. Centre for Blistering Diseases, Department of Dermatology, University Medical Centre Groningen, University of Groningen, P.O. Box 30.001, 9700 RB, Groningen, The Netherlands
  • 刊物类别:Biomedical and Life Sciences
  • 刊物主题:Biomedicine
    Human Genetics
    Proteomics
    Molecular Medicine
  • 出版者:Springer Berlin / Heidelberg
  • ISSN:1432-0878
文摘
The hemidesmosome is a specialized transmembrane complex that mediates the binding of epithelial cells to the underlying basement membrane. In the skin, this multiprotein structure can be regarded as the chief adhesion unit at the site of the dermal-epidermal junction. Focal adhesions are additional specialized attachment structures located between hemidesmosomes. The integrity of the skin relies on well-assembled and functional hemidesmosomes and focal adhesions (also known as integrin adhesomes). However, if these adhesion structures are impaired, e.g., as a result of circulating autoantibodies or inherited genetic mutations, the mechanical strength of the skin is compromised, leading to blistering and/or tissue inflammation. A particular clinical presentation emerges subject to the molecule that is targeted. None of these junctional complexes are simply compounds of adhesion molecules; they also play a significant role in signalling pathways involved in the differentiation and migration of epithelial cells such as during wound healing and in tumour invasion. We summarize current knowledge about hereditary and acquired blistering diseases emerging from pathologies of the hemidesmosome and its neighbouring proteins as components of the dermal-epidermal junction.

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