Defective DNA-dependent protein kinase activity is linked to V(D)J recombination and DNA repair defects associated with the murine scid mutation
Murine cells homozygous for the severe combined immune deficiency mutation ( scid) and V3 mutant hamster cells fall into the same complementation group and show similar defects in V(D)J recombination and DNA double-stranded break repair. Here we show that both cell types lack DNA-dependent protein k...
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Veröffentlicht in: | Cell 1995-03, Vol.80 (5), p.813-823 |
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Sprache: | eng |
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Zusammenfassung: | Murine cells homozygous for the severe combined immune deficiency mutation (
scid) and V3 mutant hamster cells fall into the same complementation group and show similar defects in V(D)J recombination and DNA double-stranded break repair. Here we show that both cell types lack DNA-dependent protein kinase (DNA-PK) activity owing to defects in DNA-PK
cs, the catalytic subunit of this enzyme. Furthermore, we demonstrate that yeast artificial chromosomes containing the DNA-PK
cs, gene complement both the DNA repair and recombination deficiencies of V3 cells, and we conclude that DNA-PK
cs, is encoded by the
XRCC7 gene. As DNA-PK binds to DNA ends and is activated by these structures, our findings provide novel insights into V(D)J recombination and DNA repair processes. |
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ISSN: | 0092-8674 1097-4172 |
DOI: | 10.1016/0092-8674(95)90360-7 |