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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Bibliographic Details
Published in:Cells (Basel, Switzerland) Switzerland), 2024-02, Vol.13 (5), p.402
Main Authors: Zayoud, Khouloud, Chikhaoui, Asma, Kraoua, Ichraf, Tebourbi, Anis, Najjar, Dorra, Ayari, Saker, Safra, Ines, Kraiem, Imen, Turki, Ilhem, Menif, Samia, Yacoub-Youssef, Houda
Format: Article
Language:English
Subjects:
Adaptive immunity
Aged
Aging
Analysis
Antigens
Biological markers
Biomarkers
Blood
CD27 antigen
CD28 antigen
CD8 antigen
CD8-Positive T-Lymphocytes
Chemokines
Cockayne Syndrome
Cytokines
Development and progression
Diabetes
Diagnosis
DNA repair
Flow cytometry
Genotype & phenotype
Geriatrics
Health aspects
Hemoglobin
Hereditary diseases
Humans
Immune status
Immune system
Immunity
Immunosenescence
Infections
inflammaging
Inflammation
Influenza
Lymphocytes T
Monocytes
Mortality
Mutation
Older people
Pneumonia
progeroid syndrome
Respiratory tract infection
Risk factors
Senescence
Statistical analysis
Tumor necrosis factor-TNF
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Summary: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.
ISSN:2073-4409
2073-4409
DOI:10.3390/cells13050402