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Phenotypic and Functional Analysis of Activated Regulatory T Cells Isolated from Chronic Lymphocytic Choriomeningitis Virus-infected Mice
Published on: June 22, 2016
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Regulatory T cell features in chronic granulomatous disease
A van de Geer1, E Cuadrado2, M C Slot2
1Department of Blood Cell Research, Sanquin Research, Amsterdam UMC, University of Amsterdam, Amsterdam, the Netherlands.
Clinical and Experimental Immunology
|March 30, 2019
Summary
Regulatory T cells (Tregs) are not generally deficient in Chronic Granulomatous Disease (CGD). However, a specific CGD subtype shows reduced effector Tregs, though their function remains normal, suggesting Tregs are not the primary cause of CGD autoimmunity.
Area of Science:
- Immunology
- Primary Immunodeficiency
- T cell biology
Background:
- Chronic Granulomatous Disease (CGD) is a primary immunodeficiency characterized by severe infections and autoinflammation/autoimmunity.
- The pathophysiology of CGD-associated autoinflammation/autoimmunity is not fully understood.
- Regulatory T cells (Tregs) are crucial for immune homeostasis, and their induction can be influenced by reactive oxygen species (ROS) produced by the NADPH oxidase system, which is defective in CGD.
Purpose of the Study:
- To investigate potential numerical or functional deficiencies in regulatory T cells (Tregs) in patients with Chronic Granulomatous Disease (CGD).
- To explore whether specific CGD subtypes, particularly gp91phox-, p47phox-, and p40phox-deficient forms, exhibit distinct Treg abnormalities.
- To determine if Treg alterations correlate with the autoinflammation/autoimmunity observed in CGD.
Main Methods:
- Comparative analysis of Treg numbers and suppressive function in CGD patients versus healthy controls.
- Subgroup analysis of Tregs based on specific genetic defects (gp91phox, p47phox, p40phox).
- Assessment of Treg marker expression (CD25, ICOS, Helios, CTLA-4, GITR) and in vitro functional assays.
Main Results:
- Treg numbers and suppressive capacities were comparable between CGD patients and controls, except for a decrease in effector Tregs (eTregs) in gp91phox-deficient CGD patients.
- Expression of key Treg markers did not reveal differences in Treg functionality or activation state across patient groups.
- No correlation was found between eTreg numbers and the clinical phenotype of CGD patients.
Conclusions:
- The primary finding is a reduction in circulating effector Tregs (eTregs) specifically in gp91phox-deficient CGD, a subtype most affected by autoinflammation/autoimmunity.
- Despite this numerical difference, patient-derived Tregs exhibited normal phenotype and suppressor activity in vitro, indicating preserved functionality.
- These findings suggest that Tregs are unlikely to be the primary drivers of autoinflammation/autoimmunity in Chronic Granulomatous Disease.
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