Cells (Feb 2024)

Immunity in the Progeroid Model of Cockayne Syndrome: Biomarkers of Pathological Aging

  • Khouloud Zayoud,
  • Asma Chikhaoui,
  • Ichraf Kraoua,
  • Anis Tebourbi,
  • Dorra Najjar,
  • Saker Ayari,
  • Ines Safra,
  • Imen Kraiem,
  • Ilhem Turki,
  • Samia Menif,
  • Houda Yacoub-Youssef

DOI
https://doi.org/10.3390/cells13050402
Journal volume & issue
Vol. 13, no. 5
p. 402

Abstract

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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.

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