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Vol. 14, No. 20, pp. 2576-2580, October 15, 2000
1 Department of Genetics, St Jude Children's Research
Hospital, Memphis, Tennessee 38105, USA; 2 Imperial Cancer
Research Fund, Clare Hall Laboratories, South Mimms, Herts EN6 3LD, UK
Ataxia telangiectasia results from mutations of ATM and is
characterized by severe neurodegeneration and defective responses to
DNA damage. Inactivation of certain DNA repair genes such as DNA ligase
IV results in massive neuronal apoptosis and embryonic lethality in the
mouse, indicating the occurrence of endogenously formed DNA
double-strand breaks during nervous system development. Here we report
that Atm is required for apoptosis in all areas of the DNA ligase
IV-deficient developing nervous system. However, Atm deficiency failed
to rescue deficits in immune differentiation in DNA ligase IV-null
mice. These data indicate that ATM responds to endogenous DNA lesions
and functions during development to eliminate neural cells that have
incurred genomic damage. Therefore, ATM could be important for
preventing accumulation of DNA-damaged cells in the nervous system that
might eventually lead to the neurodegeneration observed in ataxia telangiectasia.
[Key Words: ATM; DNA ligase IV; apoptosis; neurodegeneration; ataxia telangiectasia]
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