An apoptosis-inducing genotoxin differentiates heterozygotic carriers for Werner helicase mutations from wild-type and homozygous mutants

Immortalized B lymphocytes from Werner syndrome subjects are shown to be hypersensitive to 4-nitroquinoline-1-oxide (4NQO), supporting earlier work on T lymphocytes. We also show that B cell lines from clinically normal heterozygous carriers exhibit sensitivities to this genotoxic agent, which are i...

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Bibliographic Details
Published in:Human genetics 1997-12, Vol.101 (2), p.121-125
Main Authors: OGBURN, C. E, OSHIMA, J, POOT, M, CHEN, R, HUNT, K. E, GOLLAHON, K. A, RABINOVITCH, P. S, MARTIN, G. M
Format: Article
Language:English
Subjects:
4-Nitroquinoline-1-oxide - pharmacology
Apoptosis - genetics
B-Lymphocytes - drug effects
Biological and medical sciences
Cell Line
Complex syndromes
DNA Helicases - genetics
Dose-Response Relationship, Drug
Exodeoxyribonucleases
Heterozygote
Homozygote
Humans
Medical genetics
Medical sciences
Mutagens - pharmacology
Mutation
Phenotype
RecQ Helicases
Werner Syndrome - enzymology
Werner Syndrome - genetics
Werner Syndrome Helicase
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Summary:Immortalized B lymphocytes from Werner syndrome subjects are shown to be hypersensitive to 4-nitroquinoline-1-oxide (4NQO), supporting earlier work on T lymphocytes. We also show that B cell lines from clinically normal heterozygous carriers exhibit sensitivities to this genotoxic agent, which are intermediate to those of wild-type and homozygous mutants. 4NQO is shown to induce an apoptotic response. These data encourage research on DNA repair with such cell lines and raise the question of an enhanced sensitivity of the relatively prevalent heterozygous carriers to certain environmental genotoxic agents.
ISSN:0340-6717
1432-1203
DOI:10.1007/s004390050599