Abstract
Evaluation of: Nishie W, Sawamura D, Goto M et al. Humanuzation of autoantigen. Nat. Med. 13, 378–383 (2007).
The development of a conventional passive transfer model of bullous pemphigoid (BP) is made impossible by the fact that BP antibodies that react with the immunodominant and potentially pathogenic epitope within the NC16A region fail to cross-react with the murine form of COL17. A recent paper by Nishie et al. describes one of the first successful murine models of organ-specific autoimmunity based on genetic modification and ‘humanization’ of the autoantigen (COL17). A COL17 knockout mouse (a murine equivalent of non-Herlitz junctional epidermolysis bullosa) was rescued by the human ortholog, which made it susceptible to a disease resembling BP on passive transfer of BP patients’ sera. The data presented provides in vivo evidence of the pathogenicity of circulating anti-NC16A autoantibodies from patients with BP.
Financial disclosure
The authors have no relevant financial interests related to this manuscript, including employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.