Anti-collagen XVII single-chain Fv antibody blocks the autoimmune reaction mediated by pathogenic autoantibodies in bullous pemphigoid

Yan, Wu; Sun, Nana; Dang, Erle; Jin, Liang; Zhang, Wei; Yang, Luting; Wang, Gang

doi:10.1016/j.jdermsci.2013.05.010

Cited by 2 publications

(1 citation statement)

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“…Complement-based therapeutics have been considered for the treatment of BP 33–35 . A genetically engineered Fab antibody and scfv against BP180-NC16A IgG subtype without complement activation were reported to have potential therapeutic value in BP 36 . However, application of complement regulation as a therapeutic strategy has not been studied in BP.…”

Section: Discussionmentioning

confidence: 99%

Dysregulation of mCD46 and sCD46 contribute to the pathogenesis of bullous pemphigoid

Qiao

Dang

Cao

et al. 2017

Sci Rep

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Bullous pemphigoid (BP) is an autoimmune bullous disease caused by autoantibodies against BP180 in the epidermal basement membrane. Autoantibody-mediated complement activation is an important process in BP pathogenesis. CD46, a crucial complement regulatory protein in the complement activation, has been reported to be involved in several autoimmune diseases. In the present study, we investigated whether CD46 plays a role in BP development. We found that sCD46 expression was significantly increased in the serum and blister fluids of BP patients and correlated with the levels of anti-BP180 NC16A antibody and C3a. Otherwise, the level of mCD46 was decreased in lesions of BP patients, whereas the complement activation was enhanced. We also found that CD46 knockdown in HaCaT human keratinocytes enhanced autoantibody-mediated complement activation. Importantly, exogenous CD46 blocked complement activation in both healthy skin sections and keratinocytes induced by exposure to pathogenic antibodies from BP patients. These data suggest that CD46 deficiency is an important factor in BP pathogenesis and that increasing CD46 levels might be an effective treatment for BP.

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Section: Discussionmentioning

confidence: 99%