Abstract
Bullous pemphigoid (BP) is an autoimmune blistering skin disease associated with autoantibodies against two hemidesmosomal proteins, BP180 (type XVII collagen) and BP230. As the pathogenic relevance of antibodies against the immunodominant NC16A domain of BP180 has been clearly demonstrated, specific removal of these antibodies should be a rational therapeutic approach. Here, we evaluated three recombinant forms of bacterially produced BP180 NC16A, a monomer, trimer, and tetramer, together with different matrices for their efficacy to specifically adsorb autoantibodies from BP plasma samples. An adsorber consisting of NC16A-trimer coupled to NHS-activated Sepharose 4 Fast Flow revealed satisfying adsorption rates and a high specificity. The NC16A-trimer adsorber was regenerable and autoclavable. It has the potential to be used for specific immunoadsorption to treat severe and refractory BP and other pemphigoid diseases associated with BP180 NC16A reactivity.
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This work was supported by the European Regional Development Fund (project no. 122-09-017) and the Excellence Cluster Inflammation@Interfaces (DFG EXC306/1 and 2).
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MM, KE, LK, CP are employees, WS and WSch are board members of EUROIMMUN AG. ES and DZ have a scientific cooperation with EUROIMMUN.
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C. Probst and E. Schmidt contributed equally.
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Mersmann, M., Dworschak, J., Ebermann, K. et al. Immunoadsorber for specific apheresis of autoantibodies in the treatment of bullous pemphigoid. Arch Dermatol Res 308, 31–38 (2016). https://doi.org/10.1007/s00403-015-1606-7
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DOI: https://doi.org/10.1007/s00403-015-1606-7