Increased Expression of B Lymphocyte Induced Maturation Protein 1 (BLIMP1) in Patients with Common Variable Immunodeficiency (CVID)

  • Shockrollah Farrokhi Department of Immunology, Faculty of Medicine, Boushehr University of Medical Sciences, Boushehr, Iran
  • Faezeh Abbasi-rad Department of Immunology, Faculty of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran
  • Nafiseh Esmaeil Department of Immunology, Faculty of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran
  • Roya Sherkat Acquired Immunodeficiency Research Center, Isfahan University of Medical Sciences, Isfahan, Iran
  • Reza Yazdani Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center Hospital, Tehran University of Medical Sciences, Tehran, Iran
  • Sanaz Afshar-Ghasemlou Department of Immunology, Faculty of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran
  • Saba Fekrvand Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center Hospital, Tehran University of Medical Sciences, Tehran, Iran
  • Mazdak Ganjalikhani-Hakemi Mail Department of Immunology, Faculty of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran AND Acquired Immunodeficiency Research Center, Isfahan University of Medical Sciences, Isfahan, Iran
Keywords:
B-cell lymphoma 6 protein (BCL6), Common variable immunodeficiency (CVID), Plasma cell

Abstract

Common variable immunodeficiency (CVID) is a primary immune deficiency disorder characterized by a failure in B cell differentiation, impaired immunoglobulin production,and defect in response to vaccines. As a result of defective B cell maturation and differentiation in CVID, the affected patients commonly present with reduced numbers of memory B cell and antibody-secreting plasma cells. B-cell lymphoma 6 protein (BCL6) and B lymphocyte induced maturation protein 1 (BLIMP1) molecules are two important transcription factors that have key roles in the maturation of B cells to plasma cells. Hence, in the current survey, we aimed to evaluate the mRNA and protein expression levels of BCL6 and BLIMP1 in B lymphocytes isolated from peripheral blood in CVID patients.
We collected blood samples from 12 CVID patients and 12 healthy controls. We isolated peripheral blood mononuclear cells (PBMCs) using Ficoll density gradient separation. Then, CD19+ B cells were purified using MACS. The protein expression and transcriptional level of BCL6 and BLIMP1 were respectively measured using flow cytometry and real-time PCR.
Our results showed that the BLIMP1 mRNA expression, as well as BLIMP1 protein expression, were significantly higher in CVID patients compared to control subjects (p=0.009 and p=0.007, respectively). However, we found no significant difference in mRNA and protein expression of BCL6 between patients and healthy controls.
According to our findings, increased mRNA and protein expression levels of BLIMP1 could be involved in defective maturation of B cells in patients with CVID and elucidate mechanistic insights into the pathogenesis of this disorder.

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Published
2020-08-25
How to Cite
1.
Farrokhi S, Abbasi-rad F, Esmaeil N, Sherkat R, Yazdani R, Afshar-Ghasemlou S, Fekrvand S, Ganjalikhani-Hakemi M. Increased Expression of B Lymphocyte Induced Maturation Protein 1 (BLIMP1) in Patients with Common Variable Immunodeficiency (CVID). Iran J Allergy Asthma Immunol. 19(4):435-444.
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Original Article(s)