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    Cellular radiosensitivity in human severe-combined-immunodeficiency (SCID) syndromes.

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    Authors
    Sproston, Anthony R
    West, Catharine M L
    Hendry, Jolyon H
    Affiliation
    Department of Experimental Radiation Oncology, Paterson Institute for Cancer Research, Christie Hospital (NHS) Trust, Manchester, UK.
    Issue Date
    1997-01
    
    Metadata
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    Abstract
    PURPOSE: The aim of the work was to establish to what extent a variety of human severe-combined-immunodeficiency (SCID) disorders are associated with in vitro cellular hypersensitivity to ionizing radiation. MATERIALS AND METHODS: A study was made of fibroblast strains established from individuals with adenosine deaminase deficiency, T(-)B(-) SCID, Omenn's syndrome and a SCID heterozygote. For comparison, an assessment was also made of the radiosensitivity of a series of fibroblast strains derived from: normal donors, a patients with ataxia-telangiectasia (A-T) and an A-T heterozygote. Radiosensitivity was determined using a clonogenic assay following both high (HDR) and low (LDR) dose-rate irradiation. RESULTS: Following HDR irradiation, the fibroblast strains derived from the different human SCID disorders displayed a wide range of radiosensitivity: the adenosine deaminase deficiency cells were similar in radiosensitivity to normal fibroblasts, T(-)B(-) cells were as hypersensitive to radiation as A-T cells and the Omenn's syndrome cells showed intermediate radiosensitivity. However, whereas all four normal cell strains studied showed significant LDR sparing, none of the SCID fibroblasts did. CONCLUSIONS: These data indicate that human SCID is variable in terms of radiosensitivity depending on the particular defect. In addition, the lack of LDR sparing of radiation-induced damage suggests the involvement of some form(s) of DNA repair defect in all the human SCID syndromes.
    Citation
    Cellular radiosensitivity in human severe-combined-immunodeficiency (SCID) syndromes. 1997, 42 (1):53-7 Radiother Oncol
    Journal
    Radiotherapy and Oncology
    URI
    http://hdl.handle.net/10541/95256
    DOI
    10.1016/S0167-8140(96)01865-8
    PubMed ID
    9132827
    Type
    Article
    Language
    en
    ISSN
    0167-8140
    ae974a485f413a2113503eed53cd6c53
    10.1016/S0167-8140(96)01865-8
    Scopus Count
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