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Evaluation of Expression of LRBA and CTLA-4 Proteins in Common Variable Immunodeficiency Patients.
- Source :
-
Immunological Investigations . Feb 2022, Vol. 51 Issue 2, p381-394. 14p. - Publication Year :
- 2022
-
Abstract
- Common variable immunodeficiency (CVID) is a primary immunodeficiency disease with a heterogeneous genetic background. Lipopolysaccharide-responsive beige-like anchor (LRBA), as well as cytotoxic T-lymphocyte-associated protein 4 (CTLA-4), have important regulatory roles in the immune responses. Here, we have investigated the expression of LRBA and CTLA-4 proteins in CVID patients with at least one presentation of early-onset occurrence, autoimmunity, or enteropathy. In this study, 20 newly diagnosed CVID patients without infection only phenotype, and ten healthy individuals were enrolled. The expressions of LRBA and CTLA-4 proteins were assessed by western blotting and flow cytometry, respectively. The patients were divided into two groups of autoimmunity-positive (11 cases) and autoimmunity-negative (9 patients). LRBA and CTLA-4 expressions were significantly lower in autoimmune-positive patients than in healthy individuals (P =.03 and P =.03, respectively). Autoimmune-negative patients had lower expression of LRBA and CTLA-4 than the control group, although it was not significant. There was a positive correlation between the expressions of LRBA and CTLA-4 in both groups of patients (P <.05). Furthermore, the highest frequency of LRBA (85.7%) and CTLA-4 (71.4%) defects was detected in those with concomitant presence of autoimmunity, enteropathy, and early-onset occurrence. Concurrent presence of autoimmunity, enteropathy, and early-onset occurrence in CVID patients could be indicative of a lack of expression in LRBA and CTLA-4 proteins. This could be helpful in early diagnosis and initiation of appropriate treatment in these patients prior to genetic confirmation. [ABSTRACT FROM AUTHOR]
Details
- Language :
- English
- ISSN :
- 08820139
- Volume :
- 51
- Issue :
- 2
- Database :
- Academic Search Index
- Journal :
- Immunological Investigations
- Publication Type :
- Academic Journal
- Accession number :
- 155003062
- Full Text :
- https://doi.org/10.1080/08820139.2020.1833029