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G2-Lymphocyte Chromosomal Radiosensitivity in Patients With Lps Responsive Beige-Like Anchor Protein (Lrba) Deficiency Publisher Pubmed



Mozdarani H1 ; Kiaee F2, 3 ; Fekrvand S2 ; Azizi G4 ; Yazdani R2 ; Zakidizaji M1 ; Mozdarani S5 ; Mozdarani S5 ; Nosrati H6 ; Abolhassani H2, 7 ; Aghamohammadi A2
Authors
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Authors Affiliations
  1. 1. Faculty of Medical Sciences, Department of Medical Genetics, Tarbiat Modares University, Terhran, Iran
  2. 2. Research Center for Immunodeficiencies, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran
  3. 3. Department of Medical Immunology, School of Medicine, Shahid Beheshti University of Medical Sciences, Tehran, Iran
  4. 4. Non-communicable Diseases Research Center, Alborz University of Medical Sciences, Karaj, Iran
  5. 5. Cytogenome Medical Genetics laboratory, Chamran Medical Building, Tehran, Iran
  6. 6. Radiotherapy Department, Cancer Institute, Imam Khomeini Hospital, Tehran, Iran
  7. 7. Division of Clinical Immunology, Department of Laboratory Medicine, Karolinska Institutet at the Karolinska University Hospital Huddinge, Stockholm, Sweden

Source: International Journal of Radiation Biology Published:2019


Abstract

Lipopolysaccharide-responsive, beige-like anchor protein (LRBA) deficiency is an autosomal recessive primary immunodeficiency disease characterized by a CVID-like phenotype, particularly severe autoimmunity and inflammatory bowel disease. This study was undertaken to evaluate radiation sensitivity in 11 LRBA-deficient patients. Therefore, stimulated lymphocytes of the studied subjects were exposed to a low dose γ-radiation (100 cGy) in the G2 phase of the cell cycle and chromosomal aberrations were scored. Lymphocytes of age-sex matched healthy individuals used in the same way as controls. Based on the G2-assay, six (54.5%) of the patients had higher radiosensitivity score comparing to the healthy control group, forming the radiosensitive LRBA-deficient patients. This chromosomal radiosensitivity showed that these patients are predisposed to autoimmunity and/or malignancy, and should be protected from unnecessary diagnostic and therapeutic procedures using ionizing radiation and exposure to other DNA damaging agents. © 2019, Copyright © 2019 Taylor & Francis Group LLC.
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