scholarly journals Immunophenotyping of putative human B1 B cells in healthy controls and common variable immunodeficiency (CVID) patients

2012 ◽  
Vol 170 (3) ◽  
pp. 333-341 ◽  
Author(s):  
O. Suchanek ◽  
R. Sadler ◽  
E. A. Bateman ◽  
S. Y. Patel ◽  
B. L. Ferry
Keyword(s):  
B Cells ◽  
Common Variable Immunodeficiency ◽  
Healthy Controls ◽  
Common Variable ◽  
B1 B Cells

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

2020 ◽  
Author(s):  
Shockrollah Farrokhi ◽  
Faezeh Abbasi-rad ◽  
Nafiseh Esmaeil ◽  
Roya Sherkat ◽  
Reza Yazdani ◽  
...  
Keyword(s):  
B Cells ◽  
Protein Expression ◽  
B Cell ◽  
Common Variable Immunodeficiency ◽  
B Lymphocyte ◽  
Plasma Cells ◽  
Healthy Controls ◽  
Maturation Protein ◽  
Mrna And Protein Expression ◽  
Common Variable

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.

scholarly journals Evaluation of MicroRNA-125b-5p and Transcription Factors BLIMP1 and IRF4 Expression in Unsolved Common Variable Immunodeficiency Patients

2021 ◽  
Author(s):  
Zahra Hamidi Esfahani ◽  
Reza Yazdani ◽  
Sepideh Shahkarami ◽  
Fateme Babaha ◽  
Hassan Abolhassani ◽  
...  
Keyword(s):  
Transcription Factors ◽  
B Cells ◽  
B Lymphocytes ◽  
Common Variable Immunodeficiency ◽  
Regulatory Protein ◽  
Genetic Defect ◽  
Genetic Diagnosis ◽  
Healthy Controls ◽  
Gene Expressions ◽  
Common Variable

Common variable immunodeficiency (CVID) is the most prevalent form of symptomatic primary humoral immunodeficiencies characterized by failure in the final differentiation of B lymphocytes. The majority of CVID cases have no identified genetic defect, and epigenetic alteration could be involved in the pathogenesis of CVID. Hence, we aimed to evaluate the expression of hsa-miR-125b-5p -and, B lymphocyte-induced maturation protein-1(BLIMP-1) and interferon regulatory protein-4 (IRF-4) in a group of CVID patients with no definitive genetic diagnosis in comparison with healthy individuals. Ten CVID patients (all known genes excluded) and 10 age and sex-matched healthy controls participated in the study. B lymphocytes were isolated and expression of miR-125b-5p, IRF4, and BLIMP1 were evaluated by real-time polymerase chain reaction (RT-PCR). Moreover, B cell subsets were analyzed by flow cytometry. The results showed that the relative expression of miR-125b-5p in CVID patients was increased while it was decreased for the BLIMP1 and IRF4 transcription factors compared with the healthy controls. Although a reduction was observed in switched and non-switched memory B cells among all high-miR patients, these subsets were decreased in patients with normal miR expression (71.0% and 85.0%, respectively). Our results suggest that overexpression of miR-125b-5p affects the terminal differentiation of B cells in a selected group of CVID patients by downregulating the BLIMP-1 gene and more intensively for the IRF-4 gene expressions.  

scholarly journals Oxidative stress in common variable immunodeficiency

2021 ◽  
Vol 19 ◽  
pp. 205873922110024
Author(s):  
Sevgen Tanir Basaranoglu ◽  
Sukru Cekic ◽  
Emine Kirhan ◽  
Melahat Dirican ◽  
Sara S. Kilic
Keyword(s):  
Oxidative Stress ◽  
Common Variable Immunodeficiency ◽  
Chronic Inflammatory Demyelinating Polyneuropathy ◽  
Infectious Complications ◽  
Clinical Syndrome ◽  
University Hospital ◽  
Unknown Etiology ◽  
Healthy Controls ◽  
Oxidative Stress Biomarkers ◽  
Common Variable

Common variable immunodeficiency (CVID) is a heterogenous group of immunologic disorders of unknown etiology. Alterations of the normal cellular balance due to an increase in reactive oxygen species and/or decrease in antioxidant defense may lead to increased oxidative stress. We aimed to evaluate the levels of oxidative stress biomarkers in patients with CVID who had different presentations. We investigated the serum catalase (CAT), erythrocyte superoxide dismutase (SOD), erythrocyte reduced glutathione as antioxidants and serum malondialdehyde levels as lipid peroxidation marker in patients with CVID in Uludag University Hospital Department of Pediatric Allergy and Immunology’s outpatient clinics. In the analysis, there were 21 patients and 27 matched healthy controls. The median levels of CAT in patients with CVID was significantly lower than in healthy controls ( p = 0.04). Among the patients with CVID, 19% had autoimmune disease, one had Sjögren’s syndrome, one had autoimmune alopecia, one had juvenile rheumatoid arthritis, and one had chronic inflammatory demyelinating polyneuropathy. Patients with autoimmune complications had significantly lower CAT levels compared to the ones without autoimmune diseases ( p = 0.03). The patients without non-infectious complications (NICs) had lower SOD levels than the patients with NICs ( p = 0.05). The analysis of oxidative stress markers in the patients with CVID suggested a series of abnormalities in the anti-oxidant system. The clinical syndrome associations may be a useful tool for future studies to set prediction markers for the prognosis of patients with CVID.

scholarly journals Common Variable Immunodeficiency / Cvid

2018 ◽  
Vol 3 (2) ◽  
Keyword(s):  
B Cells ◽  
Common Variable Immunodeficiency ◽  
Autoimmune Disorders ◽  
Common Variable

“Variable” refers to the heterogeneousclinicalmanifestations of thisdisorder, which can include: recurrentinfections, chroniclungdisease, autoimmune disorders, also involve varioussegments of the gastrointestinaltract and a heightenedsusceptibility to lymphoma. Itis a primaryimmunodeficiencythataffects 1 in 50,000 peopleworldwide. Itischaracterized by reducedimmunoglobulinserumlevels and absent or impairedantibody production. The pathogenic of CVID isnotknown; however, therehavebeennumerousassociatedlaboratoryfindingsincludingnumerousmutations in the genesresult in dysfunctional B cells. The mostfrequentmutationsoccur in the TNFRSF13B gene. Genesthathavebeenimplicated in monogenic CVID include ICOS, TNFRSF13B (TACI), TNFRSF13C (BAFF-R), TNFSF12 (TWEAK), CD19, CD81, CR2 (CD21), MS4A1 (CD20), TNFRSF7 (CD27), IL21, IL21R, LRBA, CTLA4, PRKCD, PLCG2, NFKB1, NFKB2, PIK3CD, PIK3R1, VAV1, RAC2, BLK, IKZF1 (IKAROS) and IRF2BP2 [1]. In addition, thereisevidence of complexinheritanceratherthan a monogenic CVID.

scholarly journals Common variable immunodeficiency patients display elevated plasma levels of granulocyte activation markers elastase and myeloperoxidase

2019 ◽  
Vol 33 ◽  
pp. 205873841984338 ◽  
Author(s):  
Jiří Litzman ◽  
Zita Chovancová ◽  
Petr Bejdák ◽  
Marek Litzman ◽  
Zdeněk Hel ◽  
...  
Keyword(s):  
Common Variable Immunodeficiency ◽  
Plasma Levels ◽  
Cell Activation ◽  
Acute Infection ◽  
Stable State ◽  
Enzyme Linked Immunosorbent Assay ◽  
Healthy Controls ◽  
Activation Markers ◽  
Common Variable ◽  
Ivig Administration

Common variable immunodeficiency disorders (CVIDs) represent a group of primary immunodeficiency diseases characterized by hypogammaglobulinemia and dysfunctional immune response to invading pathogens. Previous studies have indicated that CVID is associated with microbial translocation and systemic myeloid cell activation. The goal of this study was to determine whether patients with CVID display elevated systemic levels of markers of granulocyte activation and whether the levels are further influenced by intravenous immunoglobulin (IVIg) infusions. The plasma levels of granulocyte activation markers elastase and myeloperoxidase were determined using enzyme-linked immunosorbent assay (ELISA) in 46 CVID patients and 44 healthy controls. All CVID patients were in a stable state with no apparent acute infection. In addition, granulocyte activation markers’ plasma levels in 24 CVID patients were determined prior to and 1 h following IVIg administration. Neutrophil elastase and myeloperoxidase plasma levels were significantly higher in CVID patients than in healthy controls. Systemic elastase levels were further increased following IVIg administration. In vitro stimulation of 13 CVID patients’ whole blood using IVIg in a therapeutically relevant dose for 2 h resulted in a significant increase in plasma elastase levels compared to unstimulated blood. The data presented here indicate that CVID is associated with chronic granulocytic activation which is further exacerbated by administering IVIg. Increased myeloperoxidase and elastase levels may contribute to associated comorbidities in CVID patients.

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