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

2.50
Hdl Handle:
http://hdl.handle.net/10541/95256
Title:
Cellular radiosensitivity in human severe-combined-immunodeficiency (SCID) syndromes.
Authors:
Sproston, Anthony R; West, Catharine M L; Hendry, Jolyon H
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.
Affiliation:
Department of Experimental Radiation Oncology, Paterson Institute for Cancer Research, Christie Hospital (NHS) Trust, Manchester, UK.
Citation:
Cellular radiosensitivity in human severe-combined-immunodeficiency (SCID) syndromes. 1997, 42 (1):53-7 Radiother Oncol
Journal:
Radiotherapy and Oncology
Issue Date:
Jan-1997
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
Appears in Collections:
All Paterson Institute for Cancer Research

Full metadata record

DC FieldValue Language
dc.contributor.authorSproston, Anthony Ren
dc.contributor.authorWest, Catharine M Len
dc.contributor.authorHendry, Jolyon Hen
dc.date.accessioned2010-03-30T13:16:31Z-
dc.date.available2010-03-30T13:16:31Z-
dc.date.issued1997-01-
dc.identifier.citationCellular radiosensitivity in human severe-combined-immunodeficiency (SCID) syndromes. 1997, 42 (1):53-7 Radiother Oncolen
dc.identifier.issn0167-8140-
dc.identifier.pmid9132827-
dc.identifier.doi10.1016/S0167-8140(96)01865-8-
dc.identifier.urihttp://hdl.handle.net/10541/95256-
dc.description.abstractPURPOSE: 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.en
dc.language.isoenen
dc.subject.meshAdenosine Deaminase-
dc.subject.meshCell Survival-
dc.subject.meshCells, Cultured-
dc.subject.meshColony-Forming Units Assay-
dc.subject.meshDose-Response Relationship, Radiation-
dc.subject.meshFibroblasts-
dc.subject.meshHumans-
dc.subject.meshRadiation Dosage-
dc.subject.meshRadiation Tolerance-
dc.subject.meshReference Values-
dc.subject.meshSevere Combined Immunodeficiency-
dc.titleCellular radiosensitivity in human severe-combined-immunodeficiency (SCID) syndromes.en
dc.typeArticleen
dc.contributor.departmentDepartment of Experimental Radiation Oncology, Paterson Institute for Cancer Research, Christie Hospital (NHS) Trust, Manchester, UK.en
dc.identifier.journalRadiotherapy and Oncologyen

Related articles on PubMed

All Items in Christie are protected by copyright, with all rights reserved, unless otherwise indicated.