title: Cellular radiosensitivity in human severe-combined-immunodeficiency (SCID) syndromes.
abstract: OBJECTIVE
  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.
  
  
  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.
authors.affiliation: Department of Experimental Radiation Oncology, Paterson Institute for Cancer Research, Christie Hospital (NHS) Trust, Manchester, UK.
authors.forename: A R
authors.initials: AR
authors.lastname: Sproston
authors.forename: C M
authors.initials: CM
authors.lastname: West
authors.forename: J H
authors.initials: JH
authors.lastname: Hendry
mesh_terms: D000243:Adenosine Deaminase / Q000172:deficiency; D002470:Cell Survival; D002478:Cells, Cultured / Q000528:radiation effects; D003114:Colony-Forming Units Assay; D004307:Dose-Response Relationship, Radiation; D005347:Fibroblasts / Q000166:cytology / Q000528:radiation effects*; D006801:Humans; D011829:Radiation Dosage; D011836:Radiation Tolerance / Q000276:immunology*; D012016:Reference Values; D016511:Severe Combined Immunodeficiency / Q000235:genetics / Q000276:immunology*
chemical_list: D000243:Adenosine Deaminase