Journal
INTERNATIONAL JOURNAL OF RADIATION BIOLOGY
Volume 95, Issue 6, Pages 680-690Publisher
TAYLOR & FRANCIS LTD
DOI: 10.1080/09553002.2019.1577570
Keywords
LRBA; immunodeficiency; G2-assay; chromosomal radiosensitivity; cancer predisposition
Funding
- Tehran University of Medical Sciences [35730-154-02-96]
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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 -radiation (100cGy) in the G(2) 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 G(2)-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.
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