| Property | Value |
|---|
|
?:abstract
|
-
Bullous pemphigoid (BP) is an autoimmune blistering disease that targets the hemidesmosomal proteins BP180 and BP230/bullous pemphigoid antigen 1e (BPAG1e). Whereas the role of anti-BP180 antibodies has been extensively characterized, the pathogenicity of anti-BPAG1e antibodies remains unclear. The purpose of this study is to elucidate the role of antibodies to BPAG1e in the experimental BP models. We generated Bpag1 conditional knockout (Bpag1-cKO) mice, where the KO of Bpag1 is restricted to keratin 5-expressing epithelial cells. Bpag1-cKO mice were immunized with the C-terminal portion of BPAG1e, and the splenocytes were injected into Rag2-/- mice intravenously. The recipient mice presented with erosion on the feet and tails. Microscopic examination showed subepidermal blisters and a linear deposition of IgG at the dermal-epidermal junction. To assess the potential role of trauma on BP development, we inflicted surface wounds on the dorsum of the Rag2-/- recipient mice after adoptive transfer. The wounded Rag2-/- mice had increased morbidity and severity of BP-like symptoms. Moreover, the depletion of B cells from splenocytes abolished a subepidermal blistering phenotype in vivo. These findings demonstrate that antibodies to BPAG1e might play a pathogenic role in causing subepidermal blistering, and external factors, including trauma, might be a trigger for BP development.
|
|
is
?:annotates
of
|
|
|
?:creator
|
|
|
?:doi
|
-
10.1016/j.jid.2020.08.031
|
|
?:doi
|
|
|
?:journal
|
-
The_Journal_of_investigative_dermatology
|
|
?:license
|
|
|
?:pmid
|
|
|
?:pmid
|
|
|
?:publication_isRelatedTo_Disease
|
|
|
is
?:relation_isRelatedTo_publication
of
|
|
|
?:source
|
|
|
?:title
|
-
Autoantibodies to BPAG1e trigger experimental bullous pemphigoid in mice.
|
|
?:type
|
|
|
?:year
|
|
![[This page as RDF]](https://research.tib.eu/covid-19/static/rdf-icon.gif){kind=icon}