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

大疱性疾病有两种类型，如自身免疫性大疱性疾病和先天性大疱性疾病。对于自身免疫性疾病，有证据表明，大疱性疾病自身抗体与抗原黏附分子结合后，角化细胞内必然发生某种信号转导。（1994年，JD，1994年大疱病年会，1995年JID正在筹备中）。据我们所知，这是世界上第一个证据。我们还发现了抗体的IgG亚类之间的功能差异。（1994年自身免疫，1995年筹备中）。总之，我们获得了大疱性疾病的新机制和免疫学新反应的第一个证据。对于先天性大疱性疾病，我们发现角蛋白丝的紊乱（1989 Arch Dermatol Res）导致角蛋白基因缺陷（1992 JID）的发现和一种新型疾病的患者（1993 Br J.Dermatol, 1995 in preparation）。对于这两种大疱性疾病，我们发现了一种新的角蛋白纤维紊乱，这种紊乱与角蛋白基因缺陷无关，而与桥粒破坏有关（1992 JDS）。换句话说，我们首次获得了桥粒与角蛋白功能关系的证据，这可能会导致一种新型粘附分子的发现。我们也得到了在大疱性疾病中重要的表皮真皮交界基底膜中蛋白多糖的角质细胞来源的证据。（1995年送至JID）。这些发现有助于理解细胞与基质的相互作用

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