Coordination Funds

PEGASUS研究小组处理天疱疮，一种罕见的皮肤和粘膜自身免疫性疾病。天疱疮是潜在的致命性，需要有效和耐受性良好的治疗策略。此外，天疱疮是一种很好的模型疾病，以更好地了解自身免疫一般天疱疮发病机制的基本机制是很好的理解。我们的小组一直在研究导致天疱疮中致病性IgG自身抗体形成的免疫机制。PEGASUS的主要焦点是T淋巴细胞，其与B淋巴细胞一起导致形成针对桥粒芯糖蛋白3的IgG自身抗体，桥粒芯糖蛋白3是上层皮肤（表皮）中的粘附分子。在天疱疮的临床前小鼠模型中，我们试图通过抑制致病性T细胞来有利地影响天疱疮的病程。这些发现将直接用于临床试验的设计，旨在提高我们对免疫系统哪些成分参与皮肤愈合和同时重建对桥粒芯蛋白3的免疫耐受性的理解。天疱疮协会的合作伙伴已经开发了实验室方法来测量和跟踪天疱疮中的致病性免疫细胞和自身抗体。进一步，我们将研究除了自身抗体与皮肤细胞结合之外，皮肤和粘膜水疱形成所需的其他因素。PEGASUS的一个标志是转化研究方法，即实验结果从实验室直接转移到诊所，无论是作为临床试验还是改进的诊断措施。我们天疱疮研究中的一些发现也可能适用于无关的自身免疫性疾病和炎症性皮肤病。

The Research Group PEGASUS deals with pemphigus, a rare autoimmune disorder of the skin and mucous membranes. Pemphigus is potentially lethal and requires efficient and well-tolerated therapeutic strategies. Moreover, pemphigus is an excellent model disease to better understand autoimmunity in general as the basic mechanisms in the pemphigus pathogenesis are well understood. Our group has been doing research on the immune mechanisms that lead to the formation of pathogenic IgG autoantibodies in pemphigus. A major focus of PEGASUS are T-lymphocytes which in concert with B-lymphocytes lead to the formation of IgG autoantibodies against desmoglein 3, an adhesion molecule in the upper skin (epidermis). In a preclinical mouse model of pemphigus, we try to favorably influence the disease course of pemphigus via inhibition of pathogenic T-cells. These findings will then go directly into the design of clinical trials aimed at improving our understanding which components of the immune system are involved in skin healing and reconstitution of immune tolerance against desmoglein 3 at the same time. Partners of the pemphigus consortium have developed laboratory methods to measure and follow-up on pathogenic immune cells and autoantibodies in pemphigus. Further on, we will study additional factors that are necessary for blister formation of skin and mucous membranes beyond autoantibody binding to skin cells. A hallmark of PEGASUS is the translational research approach, i.e. the direct transfer of experimental findings from the bench to the clinics, either as a clinical trial or improved diagnostic measure. Some of the findings evolving from our pemphigus research may also be applicable to unrelated autoimmune disorders and inflammatory skin disorders.