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The STUDIA UNIVERSITATIS BABEŞ-BOLYAI issue article summary
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Abstract: Bullous pemphigoid is an autoimmune blistering disease characterized by the presence of circulating and tissue-bound autoantibodies against two structural proteins of the hemidesmosomes, namely the bullous pemphigoid antigen of 180kDa and bullous pemphigoid antigen of 230kDa, respectively. The implication of immune players in the clinical outcome has been addressed in different studies over the past three decades and has led to the formulation of a general hypothesis that tried to explain the pathogenesis of the disease. According to this scenario, binding of autoantibodies to their target autoantigens is followed by the recruitment and activation of the complement system and further reinforced by the activation of granulocytes which release reactive oxygen species and subsequently activate proteases culminating in the enzymatic digestion of the dermal-epidermal zone. Despite our understanding in these general terms, the fine molecular processes underlying different steps of this scenario and their implications for a future targeted-therapy have yet to be ascertained. In the present study we investigated the potential of different modulators of reactive oxygen species production to induce or block tissue damage using the ex vivo model for bullous pemphigoid. Our present data indicate that despite its powerful scavenging effect of more than 85%, superoxide dismutase is not able to inhibit the blister-inducing capacity of autoantibodies. In contrast, specific inhibition of the myeloperoxidase could block the antibody-induced granulocyte-mediated dermal-epidermal separation. These results contribute to the further characterization of the pathogenic relevance of granulocytes in experimental bullous pemphigoid and should facilitate further investigations of the molecular pathogenesis of this and related diseases.
Non-standard abbreviations: AHAB, 4-aminobenzoic acid hydrazide; BP, bullous pemphigoid; DES, dermal-epidermal separation; IF, immunofluorescence; MPO, myeloperoxidase; PMA, phorbol 12-myristate 13-acetate; ROS, reactive oxygen species; SOD, superoxide dismutase.
Key words: autoimmunity, collagen XVII/BP180, myeloperoxidase, superoxide dismutase, tissue damage |
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