Proteases in pemphigoid diseases

Sho Hiroyasu, Christopher T. Turner, Katlyn C. Richardson, David J. Granville

Research output: Contribution to journalReview ArticleResearchpeer-review

20 Citations (Scopus)

Abstract

Pemphigoid diseases are a subgroup of autoimmune skin diseases characterized by widespread tense blisters. Standard of care typically involves immunosuppressive treatments, which may be insufficient and are often associated with significant adverse events. As such, a deeper understanding of the pathomechanism(s) of pemphigoid diseases is necessary in order to identify improved therapeutic approaches. A major initiator of pemphigoid diseases is the accumulation of autoantibodies against proteins at the dermal-epidermal junction (DEJ), followed by protease activation at the lesion. The contribution of proteases to pemphigoid disease pathogenesis has been investigated using a combination of in vitro and in vivo models. These studies suggest proteolytic degradation of anchoring proteins proximal to the DEJ is crucial for dermal-epidermal separation and blister formation. In addition, proteases can also augment inflammation, expose autoantigenic cryptic epitopes, and/or provoke autoantigen spreading, which are all important in pemphigoid disease pathology. The present review summarizes and critically evaluates the current understanding with respect to the role of proteases in pemphigoid diseases.

Original languageEnglish
Article number1454
Number of pages19
JournalFrontiers in Immunology
Volume10
Issue numberJUN
DOIs
Publication statusPublished - 26 Jun 2019
Externally publishedYes

Keywords

  • Bullous pemphigoid
  • Elastase
  • Epidermolysis bullosa acquisita
  • Granzyme
  • MMP
  • Mucous membrane pemphigoid
  • Pemphigoid diseases
  • Proteases

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