[HTML][HTML] Targeted disruption of the catalytic subunit of the DNA-PK gene in mice confers severe combined immunodeficiency and radiosensitivity
GE Taccioli, AG Amatucci, HJ Beamish, D Gell… - Immunity, 1998 - cell.com
GE Taccioli, AG Amatucci, HJ Beamish, D Gell, XH Xiang, MIT Arzayus, A Priestley…
Immunity, 1998•cell.comThe DNA-dependent protein kinase is a mammalian protein complex composed of Ku70,
Ku80, and DNA-PKcs subunits that has been implicated in DNA double-strand break repair
and V (D) J recombination. Here, by gene targeting, we have constructed a mouse with a
disruption in the kinase domain of DNA-PKcs, generating an animal model completely
devoid of DNA-PK activity. Our results demonstrate that DNA-PK activity is required for
coding but not for signal join formation in mice. Although our DNA-PKcs defective mice …
Ku80, and DNA-PKcs subunits that has been implicated in DNA double-strand break repair
and V (D) J recombination. Here, by gene targeting, we have constructed a mouse with a
disruption in the kinase domain of DNA-PKcs, generating an animal model completely
devoid of DNA-PK activity. Our results demonstrate that DNA-PK activity is required for
coding but not for signal join formation in mice. Although our DNA-PKcs defective mice …
Abstract
The DNA-dependent protein kinase is a mammalian protein complex composed of Ku70, Ku80, and DNA-PKcs subunits that has been implicated in DNA double-strand break repair and V(D)J recombination. Here, by gene targeting, we have constructed a mouse with a disruption in the kinase domain of DNA-PKcs, generating an animal model completely devoid of DNA-PK activity. Our results demonstrate that DNA-PK activity is required for coding but not for signal join formation in mice. Although our DNA-PKcs defective mice closely resemble Scid mice, they differ by having elevated numbers of CD4+CD8+ thymocytes. This suggests that the Scid mice may not represent a null phenotype and may retain some residual DNA-PKcs function.
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