Project: Thymic Abnormalities in Rare Immunological Diseases

Acronym	TARID (Reference Number: EJPRD19-208)
Project Topic	Many rare diseases are caused by a malfunctioning immune system attacking the patient's own tissues, but treatment options are limited by the fact that the origin of such malfunctioning is poorly understood. In the TARID project we study some of these immunological diseases, united by similar pathogenetic events and pathological findings. Common to the diseases we study is an inherited or acquired defect of AIRE-protein that normally works in the thymus organ. In the thymus AIRE takes part in the development and education of T cells, the cells responsible for cellular immunity. Our hypothesis is that when AIRE is lost in the thymus some unknown harmful mechanism allows T and B lymphocytes to collaborate and together create an abnormal response to structures found in other parts of the human body. This response results in production of antibodies reacting to body's own structures, so-called autoantibodies that are common in rare immunological diseases. We will identify key steps leading to this abnormal response, and aim to find therapeutic targets that can be manipulated to put the brakes on the pathological production of autoantibodies. The results will be tested in an AIRE-deficient animal model and validated in human tissue culture assays and patient samples. Our ultimate goal is to provide information facilitating the development and use of new modes of treatment in rare immunological diseases.
Network	EJP RD
Call	1st EJP RD Joint Transnational Call for Rare Diseases Research Project (JTC 2019)

Project partner

Number	Name	Role	Country
1	University of Helsinki	Coordinator	Finland
2	University of Helsinki	Partner	Finland
3	INSERM	Partner	France
4	INSERM	Partner	France
5	University of Tartu	Partner	Estonia
6	Universidade de Lisboa	Partner	Portugal