Immunity in the Progeroid Model of Cockayne Syndrome: Biomarkers of Pathological Aging
Cockayne syndrome (CS) is a rare autosomal recessive disorder that affects the DNA repair process. It is a progeroid syndrome predisposing patients to accelerated aging and to increased susceptibility to respiratory infections. Here, we studied the immune status of CS patients to determine potential...
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MDPI AG
2024-02-01
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| author | Khouloud Zayoud Asma Chikhaoui Ichraf Kraoua Anis Tebourbi Dorra Najjar Saker Ayari Ines Safra Imen Kraiem Ilhem Turki Samia Menif Houda Yacoub-Youssef |
| author_facet | Khouloud Zayoud Asma Chikhaoui Ichraf Kraoua Anis Tebourbi Dorra Najjar Saker Ayari Ines Safra Imen Kraiem Ilhem Turki Samia Menif Houda Yacoub-Youssef |
| author_sort | Khouloud Zayoud |
| collection | DOAJ |
| description | Cockayne syndrome (CS) is a rare autosomal recessive disorder that affects the DNA repair process. It is a progeroid syndrome predisposing patients to accelerated aging and to increased susceptibility to respiratory infections. Here, we studied the immune status of CS patients to determine potential biomarkers associated with pathological aging. CS patients, as well as elderly and young, healthy donors, were enrolled in this study. Complete blood counts for patients and donors were assessed, immune cell subsets were analyzed using flow cytometry, and candidate cytokines were analyzed via multi-analyte ELISArray kits. In CS patients, we noticed a high percentage of lymphocytes, an increased rate of intermediate and non-classical monocytes, and a high level of pro-inflammatory cytokine IL-8. In addition, we identified an increased rate of particular subtypes of T Lymphocyte CD8+ CD28− CD27−, which are senescent T cells. Thus, an inflammatory state was found in CS patients that is similar to that observed in the elderly donors and is associated with an immunosenescence status in both groups. This could explain the CS patients’ increased susceptibility to infections, which is partly due to an aging-associated inflammation process. |
| first_indexed | 2024-04-25T00:33:38Z |
| format | Article |
| id | doaj.art-b1269f6cfec544049e1cc507b336d0b9 |
| institution | Directory Open Access Journal |
| issn | 2073-4409 |
| language | English |
| last_indexed | 2024-04-25T00:33:38Z |
| publishDate | 2024-02-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Cells |
| spelling | doaj.art-b1269f6cfec544049e1cc507b336d0b92024-03-12T16:41:35ZengMDPI AGCells2073-44092024-02-0113540210.3390/cells13050402Immunity in the Progeroid Model of Cockayne Syndrome: Biomarkers of Pathological AgingKhouloud Zayoud0Asma Chikhaoui1Ichraf Kraoua2Anis Tebourbi3Dorra Najjar4Saker Ayari5Ines Safra6Imen Kraiem7Ilhem Turki8Samia Menif9Houda Yacoub-Youssef10Laboratory of Biomedical Genomics and Oncogenetics (LR16IPT05), Institut Pasteur de Tunis, Université Tunis El Manar, El Manar I, Tunis 1002, TunisiaLaboratory of Biomedical Genomics and Oncogenetics (LR16IPT05), Institut Pasteur de Tunis, Université Tunis El Manar, El Manar I, Tunis 1002, TunisiaDepartment of Neuropediatrics, National Institute of Neurology Mongi Ben Hamida, Tunis 1007, TunisiaOrthopedic and Trauma Surgery Department, Mongi Slim Hospital, La Marsa 2070, TunisiaLaboratory of Biomedical Genomics and Oncogenetics (LR16IPT05), Institut Pasteur de Tunis, Université Tunis El Manar, El Manar I, Tunis 1002, TunisiaOrthopedic and Trauma Surgery Department, Mongi Slim Hospital, La Marsa 2070, TunisiaLaboratory of Molecular and Cellular Hematology (LR16IPT07), Institut Pasteur de Tunis, Université Tunis El Manar, El Manar I, Tunis 1002, TunisiaLaboratory of Molecular and Cellular Hematology (LR16IPT07), Institut Pasteur de Tunis, Université Tunis El Manar, El Manar I, Tunis 1002, TunisiaDepartment of Neuropediatrics, National Institute of Neurology Mongi Ben Hamida, Tunis 1007, TunisiaLaboratory of Molecular and Cellular Hematology (LR16IPT07), Institut Pasteur de Tunis, Université Tunis El Manar, El Manar I, Tunis 1002, TunisiaLaboratory of Biomedical Genomics and Oncogenetics (LR16IPT05), Institut Pasteur de Tunis, Université Tunis El Manar, El Manar I, Tunis 1002, TunisiaCockayne syndrome (CS) is a rare autosomal recessive disorder that affects the DNA repair process. It is a progeroid syndrome predisposing patients to accelerated aging and to increased susceptibility to respiratory infections. Here, we studied the immune status of CS patients to determine potential biomarkers associated with pathological aging. CS patients, as well as elderly and young, healthy donors, were enrolled in this study. Complete blood counts for patients and donors were assessed, immune cell subsets were analyzed using flow cytometry, and candidate cytokines were analyzed via multi-analyte ELISArray kits. In CS patients, we noticed a high percentage of lymphocytes, an increased rate of intermediate and non-classical monocytes, and a high level of pro-inflammatory cytokine IL-8. In addition, we identified an increased rate of particular subtypes of T Lymphocyte CD8+ CD28− CD27−, which are senescent T cells. Thus, an inflammatory state was found in CS patients that is similar to that observed in the elderly donors and is associated with an immunosenescence status in both groups. This could explain the CS patients’ increased susceptibility to infections, which is partly due to an aging-associated inflammation process.https://www.mdpi.com/2073-4409/13/5/402Cockayne syndromeprogeroid syndromeinflammagingimmunosenescence |
| spellingShingle | Khouloud Zayoud Asma Chikhaoui Ichraf Kraoua Anis Tebourbi Dorra Najjar Saker Ayari Ines Safra Imen Kraiem Ilhem Turki Samia Menif Houda Yacoub-Youssef Immunity in the Progeroid Model of Cockayne Syndrome: Biomarkers of Pathological Aging Cells Cockayne syndrome progeroid syndrome inflammaging immunosenescence |
| title | Immunity in the Progeroid Model of Cockayne Syndrome: Biomarkers of Pathological Aging |
| title_full | Immunity in the Progeroid Model of Cockayne Syndrome: Biomarkers of Pathological Aging |
| title_fullStr | Immunity in the Progeroid Model of Cockayne Syndrome: Biomarkers of Pathological Aging |
| title_full_unstemmed | Immunity in the Progeroid Model of Cockayne Syndrome: Biomarkers of Pathological Aging |
| title_short | Immunity in the Progeroid Model of Cockayne Syndrome: Biomarkers of Pathological Aging |
| title_sort | immunity in the progeroid model of cockayne syndrome biomarkers of pathological aging |
| topic | Cockayne syndrome progeroid syndrome inflammaging immunosenescence |
| url | https://www.mdpi.com/2073-4409/13/5/402 |
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