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.

落叶型天疱疮（PF）是一种自身免疫性疾病，其特征是全身表面出现水疱和糜烂。抗体与抗原结合后形成水泡的机制尚未完全阐明。在这项研究中，我们观察了将来自 PF 患者的单克隆抗体注射到培养的正常人皮肤器官后超微结构的变化。结果，2小时内桥粒数量明显减少，22小时后观察到桥粒消失和细胞脱离。这一结果表明随着桥粒的减少存在细胞脱离途径。

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