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Cytoskeletal Regulation of Inflammation and Its Impact on Skin Blistering Disease Epidermolysis Bullosa Acquisita

Journal

Publisher

MDPI
DOI: 10.3390/ijms17071116

Keywords

actin cytoskeleton; inflammation; skin blistering; epidermolysis bullosa acquisita

Funding

  1. NHMRC Senior Research Fellowship (GNT) [1102617]
  2. Future Industries Institute Foundation Fellowship
  3. Excellence Cluster Inflammation at Interfaces (DFG) [EXC306/2]
  4. Research Training Groups Modulation of Autoimmunity (DFG) [GRK 1727/1]
  5. Genes Environment and Inflammation (DFG) [GRK 1743/1]
  6. Dystrophic Epidermolysis Bullosa Research Association of Australia

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Actin remodelling proteins regulate cytoskeletal cell responses and are important in both innate and adaptive immunity. These responses play a major role in providing a fine balance in a cascade of biological events that results in either protective acute inflammation or chronic inflammation that leads to a host of diseases including autoimmune inflammation mediated epidermolysis bullosa acquisita (EBA). This review describes the role of the actin cytoskeleton and in particular the actin remodelling protein called Flightless I (Flii) in regulating cellular inflammatory responses and its subsequent effect on the autoimmune skin blistering disease EBA. It also outlines the potential of an antibody based therapy for decreasing Flii expression in vivo to ameliorate the symptoms associated with EBA.

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