Gene Signature of Regulatory T Cells Isolated from Children with Selective IgA Deficiency and Common Variable Immunodeficiency
Selective IgA deficiency (SIgAD) is the most common form and common variable immunodeficiency (CVID) is the most symptomatic form of predominant antibody deficiency. Despite differences in the clinical picture, a similar genetic background is suggested. A common feature of both disorders is the occu...
| Main Authors: | , , , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
MDPI AG
2024-02-01
|
| Series: | Cells |
| Subjects: | |
| Online Access: | https://www.mdpi.com/2073-4409/13/5/417 |
| _version_ | 1797264687828566016 |
|---|---|
| author | Magdalena Rutkowska-Zapała Agnieszka Grabowska-Gurgul Marzena Lenart Anna Szaflarska Anna Kluczewska Monika Mach-Tomalska Monika Baj-Krzyworzeka Maciej Siedlar |
| author_facet | Magdalena Rutkowska-Zapała Agnieszka Grabowska-Gurgul Marzena Lenart Anna Szaflarska Anna Kluczewska Monika Mach-Tomalska Monika Baj-Krzyworzeka Maciej Siedlar |
| author_sort | Magdalena Rutkowska-Zapała |
| collection | DOAJ |
| description | Selective IgA deficiency (SIgAD) is the most common form and common variable immunodeficiency (CVID) is the most symptomatic form of predominant antibody deficiency. Despite differences in the clinical picture, a similar genetic background is suggested. A common feature of both disorders is the occurrence of autoimmune conditions. Regulatory T cells (T<sub>regs</sub>) are the major immune cell type that maintains autoimmune tolerance. As the different types of abnormalities of T<sub>reg</sub> cells have been associated with autoimmune disorders in primary immunodeficiency (PID) patients, in our study we aimed to analyze the gene expression profiles of T<sub>reg</sub> cells in CVID and SIgAD patients compared to age-matched healthy controls. The transcriptome-wide gene profiling was performed by microarray technology. As a result, we analyzed and visualized gene expression patterns of isolated population of T<sub>reg</sub> cells. We showed the differences at the gene level between patients with and without autoimmunizations. Our findings suggest that the gene signatures of T<sub>reg</sub> cells isolated from SIgAD and CVID patients differ from age-matched healthy controls and from each other, presenting transcriptional profiles enriched in innate immune or Th response, respectively. The occurrence of autoimmunity in both types of PID is associated with down-regulation of class I IFNs signaling pathways. In summary, our findings improve our understanding of T<sub>reg</sub> dysfunctions in patients with common PIDs and associated autoimmunity. |
| first_indexed | 2024-04-25T00:32:52Z |
| format | Article |
| id | doaj.art-0216ca46e5ad4d01893d7297323fb342 |
| institution | Directory Open Access Journal |
| issn | 2073-4409 |
| language | English |
| last_indexed | 2024-04-25T00:32:52Z |
| publishDate | 2024-02-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Cells |
| spelling | doaj.art-0216ca46e5ad4d01893d7297323fb3422024-03-12T16:41:38ZengMDPI AGCells2073-44092024-02-0113541710.3390/cells13050417Gene Signature of Regulatory T Cells Isolated from Children with Selective IgA Deficiency and Common Variable ImmunodeficiencyMagdalena Rutkowska-Zapała0Agnieszka Grabowska-Gurgul1Marzena Lenart2Anna Szaflarska3Anna Kluczewska4Monika Mach-Tomalska5Monika Baj-Krzyworzeka6Maciej Siedlar7Department of Clinical Immunology, Institute of Paediatrics, Jagiellonian University Medical College, Wielicka 265, 30-663 Krakow, PolandDepartment of Medical Genetics, Institute of Paediatrics, Jagiellonian University Medical College, Wielicka 265, 30-663 Krakow, PolandDepartment of Clinical Immunology, Institute of Paediatrics, Jagiellonian University Medical College, Wielicka 265, 30-663 Krakow, PolandDepartment of Clinical Immunology, Institute of Paediatrics, Jagiellonian University Medical College, Wielicka 265, 30-663 Krakow, PolandDepartment of Clinical Immunology, Institute of Paediatrics, Jagiellonian University Medical College, Wielicka 265, 30-663 Krakow, PolandDepartment of Clinical Immunology, University Children’s Hospital, Wielicka 265, 30-663 Krakow, PolandDepartment of Clinical Immunology, Institute of Paediatrics, Jagiellonian University Medical College, Wielicka 265, 30-663 Krakow, PolandDepartment of Clinical Immunology, Institute of Paediatrics, Jagiellonian University Medical College, Wielicka 265, 30-663 Krakow, PolandSelective IgA deficiency (SIgAD) is the most common form and common variable immunodeficiency (CVID) is the most symptomatic form of predominant antibody deficiency. Despite differences in the clinical picture, a similar genetic background is suggested. A common feature of both disorders is the occurrence of autoimmune conditions. Regulatory T cells (T<sub>regs</sub>) are the major immune cell type that maintains autoimmune tolerance. As the different types of abnormalities of T<sub>reg</sub> cells have been associated with autoimmune disorders in primary immunodeficiency (PID) patients, in our study we aimed to analyze the gene expression profiles of T<sub>reg</sub> cells in CVID and SIgAD patients compared to age-matched healthy controls. The transcriptome-wide gene profiling was performed by microarray technology. As a result, we analyzed and visualized gene expression patterns of isolated population of T<sub>reg</sub> cells. We showed the differences at the gene level between patients with and without autoimmunizations. Our findings suggest that the gene signatures of T<sub>reg</sub> cells isolated from SIgAD and CVID patients differ from age-matched healthy controls and from each other, presenting transcriptional profiles enriched in innate immune or Th response, respectively. The occurrence of autoimmunity in both types of PID is associated with down-regulation of class I IFNs signaling pathways. In summary, our findings improve our understanding of T<sub>reg</sub> dysfunctions in patients with common PIDs and associated autoimmunity.https://www.mdpi.com/2073-4409/13/5/417common variable immunodeficiencyselective IgA deficiencyregulatory T cellsmicroarray analysis |
| spellingShingle | Magdalena Rutkowska-Zapała Agnieszka Grabowska-Gurgul Marzena Lenart Anna Szaflarska Anna Kluczewska Monika Mach-Tomalska Monika Baj-Krzyworzeka Maciej Siedlar Gene Signature of Regulatory T Cells Isolated from Children with Selective IgA Deficiency and Common Variable Immunodeficiency Cells common variable immunodeficiency selective IgA deficiency regulatory T cells microarray analysis |
| title | Gene Signature of Regulatory T Cells Isolated from Children with Selective IgA Deficiency and Common Variable Immunodeficiency |
| title_full | Gene Signature of Regulatory T Cells Isolated from Children with Selective IgA Deficiency and Common Variable Immunodeficiency |
| title_fullStr | Gene Signature of Regulatory T Cells Isolated from Children with Selective IgA Deficiency and Common Variable Immunodeficiency |
| title_full_unstemmed | Gene Signature of Regulatory T Cells Isolated from Children with Selective IgA Deficiency and Common Variable Immunodeficiency |
| title_short | Gene Signature of Regulatory T Cells Isolated from Children with Selective IgA Deficiency and Common Variable Immunodeficiency |
| title_sort | gene signature of regulatory t cells isolated from children with selective iga deficiency and common variable immunodeficiency |
| topic | common variable immunodeficiency selective IgA deficiency regulatory T cells microarray analysis |
| url | https://www.mdpi.com/2073-4409/13/5/417 |
| work_keys_str_mv | AT magdalenarutkowskazapała genesignatureofregulatorytcellsisolatedfromchildrenwithselectiveigadeficiencyandcommonvariableimmunodeficiency AT agnieszkagrabowskagurgul genesignatureofregulatorytcellsisolatedfromchildrenwithselectiveigadeficiencyandcommonvariableimmunodeficiency AT marzenalenart genesignatureofregulatorytcellsisolatedfromchildrenwithselectiveigadeficiencyandcommonvariableimmunodeficiency AT annaszaflarska genesignatureofregulatorytcellsisolatedfromchildrenwithselectiveigadeficiencyandcommonvariableimmunodeficiency AT annakluczewska genesignatureofregulatorytcellsisolatedfromchildrenwithselectiveigadeficiencyandcommonvariableimmunodeficiency AT monikamachtomalska genesignatureofregulatorytcellsisolatedfromchildrenwithselectiveigadeficiencyandcommonvariableimmunodeficiency AT monikabajkrzyworzeka genesignatureofregulatorytcellsisolatedfromchildrenwithselectiveigadeficiencyandcommonvariableimmunodeficiency AT maciejsiedlar genesignatureofregulatorytcellsisolatedfromchildrenwithselectiveigadeficiencyandcommonvariableimmunodeficiency |