Research on the mechanism of bullous diseases---Immunological and biological studies---

点击翻译按钮获取中文摘要

There are two types of bullous diseases, such as autoimmune bullous diseases and congenital bullous diseases. As for the autoimmune diseases we could get some evidences that some sort of signal transduction must occur in the keratinocyte after the bullous diseases auto-antibodies bind to the antigens which are the adhesion molecules. (1994, JD,1994 annual meeting for bullous diseases, 1995 JID in preparation). To our knowledge, this is the first evidence in the world. Also we found the differences of the function between the IgG subclasses of the antibodies. (1994 Autoimmunity, 1995 in preparation). Taken together, We are getting the first evidences of the new mechanism for the bullous diseases and of the new type of reaction in the immunology. As for the congenital bullous diseases We found the disturbance of keratin filaments (1989 Arch Dermatol Res) which lead to the discovery of keratin gene defects(1992 JID) and a patient with new type of the disease (1993 Br J.Dermatol, 1995 in preparation). As for the matter related to the both type of the bullous diseases, we found the new type of disturbance of keratin filaments which is not related to keratin gene defects but related to desmosome destruction, (1992 JDS). In another words, We are getting the first evidences of the functional relationship between desmosome and keratin, which may lead to the discovery of a new type of adhesion molecules. We also got the evidence of keratinocyte origin of proteoglycan in the basement membrane of epidermal-dermal junction which must be important in bullous diseases. (1995 sent to JID). These findings help to understand cell to stroma interaction