Analysis of mechanism for blister formation in pemphigus foliaceus using novel monoclonal antibody with pathogenic activity

新型致病活性单克隆抗体分析落叶型天疱疮水疱形成机制

• 批准号: 20591329
• 负责人: ISHIKO Akira
• 金额: $ 3万
• 依托单位: Keio University
• 依托单位国家: 日本
• 项目类别: Grant-in-Aid for Scientific Research (C)
• 财政年份: 2008
• 资助国家: 日本
• 起止时间: 2008 至 2010
• 项目状态: 已结题
• 来源: https://kaken.nii.ac.jp/grant/KAKENHI-PROJECT-20591329/
• 关键词: 自己免疫; 免疫電顕; 水疱症; デスモソーム; 天疱瘡

Pemphigus foliaceus (PF) is an autoimmune disease characterized by blister and erosion formation over the whole body surface. The mechanism of blister formation after antibody binding to the antigen has not been fully elucidated. In this study, we observed ultrastructural change after injection of monoclonal antibody derived from the patient with PF, to the organ cultured normal human skin. As results, number of desmosomes was markedly decreased in 2 hours, and disappearance of desmosome and cell detachment were observed 22 hours afterward. This result suggested the presence of the pathway of cell detachment with the decrease of desmosome.

天疱疮叶状（PF）是一种自身免疫性疾病的特点是水泡和侵蚀形成在整个身体表面。抗体与抗原结合后形成水疱的机制尚未完全阐明。在本研究中，我们观察了注射来自PF患者的单克隆抗体后，对培养的正常人皮肤器官的超微结构变化。结果：2小时内桥粒数量明显减少，22小时后桥粒消失，细胞脱离。提示存在细胞脱离与桥粒减少的途径。

项目成果

Desmosome splitting is a primary ultrastructural change in the acantholysis of pemphigus.

桥粒分裂是天疱疮棘层松解的主要超微结构变化。

• 发表时间: 2009
• 期刊: J Dermatol Sci 54
• 作者: W. Wang;M. Amagai;A. Ishiko
• 通讯作者: A. Ishiko

Removal of amino-terminal extracellular domains of desmoglein 1 by staphylococcal exfoliative toxin is sufficient to initiate epidermal blister formation

通过葡萄球菌剥脱性毒素去除桥粒芯糖蛋白 1 的氨基末端胞外结构域足以引发表皮水疱形成

• 发表时间: 2010
• 期刊: J Dermatol Sci
• 影响因子: 4.6
• 作者: Jiageng Chen;Shoichi Hirose;Hidenori Kuwakado;and Atsuko Miyaji;Nishifuji K
• 通讯作者: Nishifuji K