Regulatory T-cell deficiency leads to pathogenic bullous pemphigoid antigen 230 autoantibody and autoimmune bullous disease

Haeberle, Stefanie; Wei, Xiaoying; Bieber, Katja; Goletz, Stephanie; Ludwig, Ralf J.; Schmidt, Enno; Enk, Alexander H.; Hadaschik, Eva

doi:10.1016/j.jaci.2018.04.006

Cited by 83 publications

(65 citation statements)

References 62 publications

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“… and Muramatsu et al. also successfully illuminated the pathogenic function of anti‐BP230 IgG autoantibodies. They demonstrated that in scurfy mice whose regulatory T cells (Treg) were defective ( Foxp 3 mutation), there was spontaneous production of IgG1 autoantibodies to BP230.…”

Section: In Vivo Studies Of Autoantibodies Against Bp230mentioning

confidence: 99%

“…Recently, Haeberle et al. and Muramatsu et al. also successfully illuminated the pathogenic function of anti‐BP230 IgG autoantibodies.…”

Section: In Vivo Studies Of Autoantibodies Against Bp230mentioning

confidence: 99%

“…Currently, most published work suggests that BP180‐specific immunoglobulin (Ig)G is the main pathogenic autoantibody in BP and the injection of anti‐BP180 IgG in animal models could lead to the key features of human BP. On the contrary, the function of the anti‐BP230 IgG antibody in the pathogenesis of BP is largely unknown, but several evidences suggested that anti‐BP230 IgG alone could cause the disease . These early studies investigating immune characteristics of BP focused on IgG reactivity.…”

Section: Introductionmentioning

confidence: 99%

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Role of BP230 autoantibodies in bullous pemphigoid

Shih

Wang

Yuan

et al. 2020

The Journal of Dermatology

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Bullous pemphigoid (BP) is an autoimmune disease associated with subepidermal blistering due to autoantibodies directed against BP180 and BP230. BP180 is currently considered as the major pathogenic autoantigen. However, previous clinical findings suggested that anti‐BP230 autoantibodies alone can cause skin lesions in animal models and many BP patients. The characteristics of BP230 and the pathogenic roles of anti‐BP230 antibodies have been proposed. First, at the molecular level, BP230 mediates the attachment of keratin intermediate filaments to the hemidesmosomal plaque and interacts with other constituents of hemidesmosomes. Second, the presence of BP230 autoantibodies may correlate with specific clinical features of BP. The immunoglobulin (Ig)G autoantibodies from BP patients react mainly against the C‐terminus of BP230, while the IgE autoantibodies are still inconclusive. Third, in vivo, autoantibodies against BP230 involved in the disease may not only induce the inflammatory response but also impair the structural stability of hemidesmosomes. This article reviews recently published work about the role of BP230 and its antibodies, including IgG and IgE, aiming to find clues of its clinical association and lay the foundation for the research on the pathogenicity of antibodies against BP230.

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Section: In Vivo Studies Of Autoantibodies Against Bp230mentioning

confidence: 99%

“…Recently, Haeberle et al. and Muramatsu et al. also successfully illuminated the pathogenic function of anti‐BP230 IgG autoantibodies.…”

Section: In Vivo Studies Of Autoantibodies Against Bp230mentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Role of BP230 autoantibodies in bullous pemphigoid

Shih

Wang

Yuan

et al. 2020

The Journal of Dermatology

View full text Add to dashboard Cite

show abstract

“…Bullous pemphigoid (BP) is an organ‐specific autoimmune disease of the skin, characterized and caused by autoantibodies targeting BP180 and/or BP230 . In the ‘classical’ and name‐giving variant, BP patients present with blisters on inflamed skin.…”

mentioning

confidence: 99%

“…Based on these findings, and on experimental data, inflammation and blistering in BP has been thought to be mediated by BP180 autoantibodies only. However, the pathogenicity of anti‐BP230 autoantibodies has recently been well documented …”

mentioning

confidence: 99%