（Pincell）ヒト化Fasリガンドマウスの作製と天疱瘡への応用：PC111抗体研究のための新たなプラットフォーム

要旨

Role of FasL in Pemphigus

• Pemphigus autoantibodies (PVIgG) trigger the release of FasL from keratinocytes (Wang et al, 2004) and high levels of FasL measured in patients’ sera (Puviani et al, 2003).
• FasL-silenced keratinocytes are protected from PVIgG-induced desmoglein-3 (Dsg3) cleavage and activation of caspase-8 induced in vitro. Moreover caspase-3 is not activated in siFasL keratinocytes upon PVIgG treatment (Lotti et al, 2018).
• Soluble FasL is essential for the process of blister formation in pemphigus (Lotti et al, 2018).
• Anti-murine FasL antibody prevents blister formation and apoptosis activation in the passive transfer pemphigus mouse model (Lotti et al, 2018).