Objective
Primary immunodeficiency diseases are rare congenital conditions. They represent an experiment of nature, demonstrating the importance of specific genes and proteins in the host defence against pathogens. In primary immuno deficiencies the susceptibility to infections often goes along with the development of autoimmune conditions. In the most prevalent human primary immunodeficiency requiring medical attention, termed Common Variable ImmunoDeficiency (CVID), the prevalence of autoimmunity is approximately 25%. Mutations in the TNF-like B cell receptor TACI, which is expressed in the germinal centre in secondary lymphoid organs, account for up to 10% of the CVID cases.
In TACI deficiency, lymphoproliferation is observed in 2/3 of the patients and autoimmune conditions in approximately 1/3 of the patients. The ligand of TACI, termed BAFF, has been shown to take a centre stage in common human autoimmune diseases such as rheumatoid arthritis and systemic lupus erythematosus. In addition to TACI, BAFF binds also to BCMA and BAFF receptor. Which of these receptors however, contribute to the development of autoimmunity has not yet been studied.
This Marie-Curie application now would like to extend the applicant's findings in TACI deficiency in CVID to other disease entities and would like to address the role of TACI in autoimmunity and ultimately address the hypothesis that some patients with an antibody deficiency syndrome may in fact suffer from an autoimmune condition in which the antibody producing cells ar e the target of autoimmunity.
This hypothesis is supported by following observations:
1. TACI-knockout mice suffer from an SLE-like autoimmune condition
2. Lymphoproliferation is observed in two thirds of patients with TACI and one third have an overt autoimmune disease
3. Autoimmune diseases are common in patients with CVID (approx. 25%) 4. B cell numbers in CVID patients are low (<6% of PBMCs) in 25% of the cases, and tend to decrease over time
Fields of science (EuroSciVoc)
CORDIS classifies projects with EuroSciVoc, a multilingual taxonomy of fields of science, through a semi-automatic process based on NLP techniques. See: The European Science Vocabulary.
CORDIS classifies projects with EuroSciVoc, a multilingual taxonomy of fields of science, through a semi-automatic process based on NLP techniques. See: The European Science Vocabulary.
- medical and health sciences clinical medicine rheumatology
- medical and health sciences medical biotechnology cells technologies stem cells
- medical and health sciences basic medicine immunology autoimmune diseases
- natural sciences biological sciences genetics mutation
- medical and health sciences clinical medicine transplantation
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Keywords
Project’s keywords as indicated by the project coordinator. Not to be confused with the EuroSciVoc taxonomy (Fields of science)
Project’s keywords as indicated by the project coordinator. Not to be confused with the EuroSciVoc taxonomy (Fields of science)
Programme(s)
Multi-annual funding programmes that define the EU’s priorities for research and innovation.
Multi-annual funding programmes that define the EU’s priorities for research and innovation.
Topic(s)
Calls for proposals are divided into topics. A topic defines a specific subject or area for which applicants can submit proposals. The description of a topic comprises its specific scope and the expected impact of the funded project.
Calls for proposals are divided into topics. A topic defines a specific subject or area for which applicants can submit proposals. The description of a topic comprises its specific scope and the expected impact of the funded project.
Call for proposal
Procedure for inviting applicants to submit project proposals, with the aim of receiving EU funding.
Procedure for inviting applicants to submit project proposals, with the aim of receiving EU funding.
FP6-2005-MOBILITY-8
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Funding Scheme
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Funding scheme (or “Type of Action”) inside a programme with common features. It specifies: the scope of what is funded; the reimbursement rate; specific evaluation criteria to qualify for funding; and the use of simplified forms of costs like lump sums.
Coordinator
LONDON
United Kingdom
The total costs incurred by this organisation to participate in the project, including direct and indirect costs. This amount is a subset of the overall project budget.