We used a modified colony survival assay to measure the sensitivity to ionizing radiation of more than 50 lymphoblastoid cell lines from normal individuals and from patients with ataxia-telangiectasia, Nijmegen breakage syndrome variants, and X-linked agammaglobulinemia. All of these disorders are associated with an increased frequency of cancer. Lymphoblastoid cell lines from patients with ataxiatelangiectasia complementation groups A, C, D, and E; ATFresno; Nijmegen breakage syndrome variants V1 and V2; and X-linked agammaglobulinemia showed marked radiosensitivity, whereas ataxia-telangiectasia heterozygotes were similar to controls. Friedreich's ataxia is not associated with increased cancer risk; lymphoblastoid cell lines from two such patients showed normal radiosensitivity. Taken together, these results suggest that some forms of X-ray sensitivity and cancer susceptibility share a common mechanism, such as an enzyme that is necessary both for the repair of radiation damage to DNA and for gene rearrangements during V(D)J recombination.

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This work was supported by the U.S. Department of Energy Grant 87ER60548, the Ataxia-Telangiectasia Medical Research Foundation, the A-T Medical Research Trust, US PHS Core Grant CA16042 to the Jonsson Comprehensive Cancer Center at UCLA, and the Italian C.N.R. Projetto Finaliizatto A.C.R.O. 92-02160-39.

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©1994 American Association for Cancer Research.
1994
American Association for Cancer Research, Inc.