NCT01167491

Study of One Protein Implicated in Wegener Disease

Activating Receptors and DAP12 Protein in Wegener's Granulomatosis

• Status: Completed
• Phase: N/A
• Study type: Interventional
• Enrollment: 138 (actual)
• Sponsor: Assistance Publique - Hôpitaux de Paris · Academic / Other
• Sex: All
• Age: 18 Years
• Healthy volunteers: Accepted

Summary

The investigators recently showed an abnormal expansion of NK-like CD4+ T cells in Wegener's granulomatosis (WG), mainly in the diffuse vasculitis presentation. These cells expressed an assortment of activating NK cell receptors and their signaling partners, in particular DAP12. The investigators hypothesize that DAP12, or a downstream signaling target of DAP12, is the missing link between the different cell components involved in WG (neutrophils, macrophages, CD4 T cells).

Detailed description

The investigators will test our hypothesis of "DAP-12 gain-of-function" by quantitative (mRNA and protein) and qualitative (DNA, signaling and cellular activation) analysis of the DAP12 signaling pathway in WG patients with localized (group 1; n=30) or diffuse WG (group 2; n=30) by comparison with patients with micro polyangitis (group 3; n=30), or with sarcoidosis ( group 4; n=30), and healthy blood donors (group 5; n=30). Blood samples will be collected during a routine medical visit. These results may help to design future therapeutic strategies based on modulation of specific intra-cellular pathways involved in the disease.

Conditions

• Wegener's Granulomatosis

Interventions

Type	Name	Description
BIOLOGICAL	Physiopathology Endpoint Classification	Blood samples will be collected during a routine medical visit.

Timeline

• Start date: 2010-05-01
• Primary completion: 2013-11-01
• Completion: 2014-09-01
• First posted: 2010-07-22
• Last updated: 2026-03-31

Locations

1 site across 1 country: France