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Endocrine, Metabolic & Immune Disorders - Drug Targets

Author(s): Fereshte Salami, Sahar Shariati, Seyed Erfan Rasouli, Samaneh Delavari, Marzieh Tavakol, Homa Sadri, Babak Asghari, Reza Yazdani, Nima Rezaei, Hassan Abolhassani and Gholamreza Azizi*

DOI: 10.2174/1871530321666211209162834

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The Effects of Stimulation with PMA/Ionomycin on CD4+ T Cell Proliferation and Surface CD4 Molecule Modulation of Patients with LRBA Deficiency and CVID with the Unsolved Genetic Defect

Page: [539 - 544] Pages: 6

  • * (Excluding Mailing and Handling)

Abstract

Background: Common variable immunodeficiency (CVID) is the most prevalent symptomatic primary immunodeficiencies. LPS-responsive beige-like anchor protein (LRBA) deficiency is a combined immunodeficiency characterized by a CVID-like phenotype. Affected patients by LRBA and CVID present a wide range of clinical manifestations, including hypogammaglobulinemia, recurrent infections, autoimmunity, as well as T cell abnormality.

Methods: The study population comprised of patients with CVID (n=10), LRBA deficiency (n=11), and healthy controls (n=12). CD4+ T cell frequency and CD4 MFI (mean fluorescence intensity) were evaluated using flow cytometry before and after stimulation with PMA/ION.

Results: The frequencies of CD4+ T cells were significantly lower in patients with LRBA deficiency than in HCs before and after treatment. In the unstimulated state, the CD4+ T cells frequency in CVID patients was significantly lower than in HCs. There were no statistically significant differences between patients and healthy individuals in CD4+ T cell proliferation. Compared to HCs, LRBA and CVID patients showed a lower CD4 MFI in unstimulated conditions. Furthermore, CD4 MFI decreased in both patients and the control group following activation.

Conclusion: Despite the reported decrease in CD4+ T cell frequency in patients with CVID and LRBA deficiency, our findings demonstrated that their CD4+ T cells have a normal proliferative response to stimuli similar to healthy individuals.

Keywords: Common variable immunodeficiency, primary immunodeficiencies, LRBA deficiency, proliferation, CD4+ T cell, stimulation.

Graphical Abstract

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