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Content archived on 2024-06-18

Natural course, pathophysiology, models for early diagnosis, prevention and innovative treatment of TNF Receptor Associated Periodic Syndrome TRAPS with application for all hereditary recurrent fevers

Objective

The TNF Receptor Associated Syndrome (TRAPS) is a rare disease of innate immunity, caused by mutations in TNFR1. It is characterised by recurrent bouts of fever and pain and mainly affects patients of European ancestry. The development of renal amyloidosis in up to 20% of cases makes it a potentially fatal disease. Corticosteroids and anti-TNF therapeutics have not been consistently effective in controlling attacks and preventing amyloidosis. EUROTRAPS is a multidisciplinary consortium, which by combining ideas, resources, and data from 7 academic participants and 2 SMEs from 6 countries, aims to gain insights into the natural course and pathophysiology of TRAPS, particularly in children. The creation of a European registry for TRAPS patients will facilitate the delineation of scores and outcome measures for diagnosis and treatment. We will examine additional pathways and genes involved in the phenotype. Kit assays will be developed to facilitate identification of disease mutations and susceptibility factors for amyloidosis and resistance to treatment. TNFR1 signalling, apoptosis and IL1 secretion abnormalities associated with TRAPS will be studied. We will also develop in vitro and humanised animal models to investigate innovative therapies. We expect that future developments in TRAPS will impact on all aspects of hereditary paediatric fevers, and thereby improve the wellbeing of patients and their families. A dedicated bipartite committee will undertake the complex task of managing this ambitious project, create a secure intranet, organise regular meetings, issue progress reports, oversee ethical, legal, financial, and gender issues (over 50% of the participants are female), adjust the workplan at each milestone, and guarantee dissemination, exploitation and protection of scientific information. The requested funding will cover salaries (over 200 person/months newly generated employments), equipment acquisition, consumables, and dissemination of knowledge.

Fields of science (EuroSciVoc)

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Keywords

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Programme(s)

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Topic(s)

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Call for proposal

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FP7-HEALTH-2007-A
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Funding Scheme

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CP-FP - Small or medium-scale focused research project

Coordinator

CENTRE HOSPITALIER UNIVERSITAIRE MONTPELLIER
EU contribution
€ 556 071,00
Address
AVENUE DU DOYEN GASTON GIRAUD 191
34000 Montpellier
France

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Region
Occitanie Languedoc-Roussillon Hérault
Activity type
Research Organisations
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Total cost

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.

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Participants (8)

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