Autoimmune Polyendocrine Syndrome Type 1 - A rare disorder of childhood as a model of autoimmunity

这个项目将分析糖尿病和其他自身免疫性疾病的机制和原因，这些疾病的免疫系统损害身体的特定器官。糖尿病是国家健康的优先事项，每20个澳大利亚人中就有一个受到自身免疫性疾病的影响。该项目将专注于最近发现的一种基因--自身免疫调节因子(AIRE)，它对于预防自身免疫性疾病至关重要。古德诺教授的团队已经证明，这种基因可以阻止免疫系统中被禁止的T淋巴细胞克隆攻击我们自己的器官。AIRE基因的遗传缺陷会导致一种毁灭性的疾病--自身免疫性多内分泌综合征1，并提供了对常见自身免疫性疾病(如1型糖尿病、甲状腺疾病、恶性贫血和爱迪生病)机制的无与伦比的洞察。通过与H·斯科特博士和欧洲的一个多学科联盟合作，古德诺教授的团队将研究AIRE基因控制的过程如何与其他基因和机制合作来预防自身免疫性疾病。这项工作将绘制出通常保护我们免受自身免疫性疾病影响的不同控制系统，并为开发治疗和预防自身免疫性疾病的新方法提供合理的基础。NHMRC的资助使澳大利亚国立大学和沃尔特和伊莱扎·霍尔研究所的两个领先的澳大利亚团队能够利用欧洲跨学科团队的一套互补技术和临床资源，扩大其世界领先的个人努力。古德诺的团队已经证明了这种澳欧合作的好处。他们发现AIRE基因在阻止被禁T细胞方面的功能依赖于与芬兰遗传学小组的密切合作，该小组由Leena Peltonen教授领导，他的团队最初是作为一个大型欧洲财团的一部分发现了AIRE基因。斯科特的团队是一个平行的欧洲-日本财团的成员，该财团同时发现了AIRE基因。EURAPS项目将建立在这些合作发现的基础上，以绘制自身免疫性疾病的机制以及如何治愈或预防它们。NHMRC为澳大利亚团队提供的资金将扩大到欧洲对整个EURAPS项目的资助的20倍。这是一项战略投资，以确保澳大利亚的卫生研究继续走在慢性病预防和治疗进步的前沿。

This project will analyse the mechanisms and causes of diabetes and other autoimmune diseases where the immune system damages particular organs of the body. Diabetes is a national health priority, and autoimmune diseases collectively affect one in every twenty Australians. The project will focus on a recently discovered gene, Autoimmune Regulator (AIRE) that is crucial for protection against autoimmune disease, which Prof Goodnow's team has shown to stop forbidden clones of T lymphocytes in the immune system from attacking our own organs. Inherited defects in the AIRE gene cause a devastating illness, Autoimmune Polyendocrine Syndrome 1, and provide an unparalleled insight into mechanisms of common autoimmune diseases such as Type 1 diabetes, thyroid diseases, pernicious anemia, and Addison's disease. By joining forces with Dr H Scott and a multidisciplinary consortium in Europe, Prof Goodnow's team will investigate how the processes controlled by the AIRE gene cooperate with other genes and mechanisms to prevent autoimmune disease. The work will chart the different control systems that normally protect us from autoimmune diseases, and provide a rational basis for developing new ways to treat and prevent autoimmune diseases. The NHMRC funding enables two leading Australian groups at The Australian National University and at the Walter and Eliza Hall Institute to amplify their world-leading individual efforts by leveraging a set of complementary technologies and clinical resources of an interdisciplinary team in Europe. Goodnow's team has already proved the benefit of this type of Australian-European collaboration. Their work discovering the function of the AIRE gene in stopping forbidden T cells depended on a close collaboration with the genetics group in Finland led by Prof Leena Peltonen, whose team had originally discovered the AIRE gene as part of a large European consortium. Scott's team was part of a parallel European-Japanese consortium that discovered the AIRE gene at the same time. The EURAPS project will build on these collaborative discoveries to chart the mechanisms of autoimmune disease and how they can be cured or prevented.The NHMRC funding for the Australian teams is amplified to a multiplier of twenty-fold by European funding for the overall EURAPS project. This represents a strategic investment to ensure Australian health research remains at the forefront of advances in prevention and treatment of chronic diseases.