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Gene Review

Xrcc6  -  X-ray repair complementing defective...

Mus musculus

Synonyms: 5'-dRP/AP lyase Ku70, 5'-deoxyribose-5-phosphate lyase Ku70, 70kDa, ATP-dependent DNA helicase 2 subunit 1, ATP-dependent DNA helicase II 70 kDa subunit, ...
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Disease relevance of Xrcc6

  • The assignment of the mouse Ku70 gene to chromosome 15 is consistent with the syntenic relationship of this gene in human (chromosome 22q13) and mouse and adds to the comparative mapping data for the genes involved in the SCID phenotype [1].
  • Ku70-deficient mice lacked mature B cells or serum immunoglobulin but, unexpectedly, reproducibly developed small populations of thymic and peripheral alpha/beta T lineage cells and had a significant incidence of thymic lymphomas [2].
  • The role of the Ku70 subunit in DNA DSB repair, hypersensitivity to ionizing radiation, and V(D)J recombination was examined in mice that lack Ku70 (Ku70(-/-)) [3].

High impact information on Xrcc6

  • We conclude that Ku70 and Ku80 may have functions in V(D)J recombination and DNA repair that are independent of DNA-PKcs [4].
  • Targeted inactivation of the Ku70 or Ku80 genes results in elevated ionizing radiation (IR) sensitivity and inability to perform both V(D)J coding-end and signal (RS)-end joining in cells, with severe growth retardation plus immunodeficiency in mice [4].
  • In association with B and T cell developmental defects, Ku70-deficient cells were severely impaired for joining of V(D)J coding and recombination signal sequences [2].
  • Growth retardation and leaky SCID phenotype of Ku70-deficient mice [2].
  • Our Ku70-deficient mice were about 50% the size of control littermates, and their fibroblasts were ionizing radiation sensitive and displayed premature senescence associated with the accumulation of nondividing cells [2].

Biological context of Xrcc6


Anatomical context of Xrcc6

  • These unanticipated features of the Ku70-deficient phenotype with respect to lymphocyte development and V(D)J recombination may reflect differential functions of the three DNA-PK components [2].
  • Mutations at multiple sites within the N-terminal two thirds of Ku80 result in loss of Ku70/80 interaction, loss of DNA end-binding activity and inability to complement Ku80 defective cell lines [9].
  • Oxidative damage and defective DNA repair is linked to apoptosis of migrating neurons and progenitors during cerebral cortex development in Ku70-deficient mice [10].
  • Mutational analysis of introduced Ku70 cDNAs into murine embryonic stem cells deleted for Ku70 (-/-) showed that mutants where heterodimerization and DNA binding functions of Ku were disrupted, also blocked the restoration of etoposide resistance [11].
  • A single band with migration properties similar to those of Ku70 was immunoprecipitated with anti-Ku antibody, using UV cross-linked complexes formed by HeLa cell nuclear extracts and an IRES-containing RNA probe [12].

Associations of Xrcc6 with chemical compounds

  • The binding required the presence of Mg(2+), implying that the sequence is a pause site for Ku70/80 translocation from a free end [7].
  • In addition, flavopiridol treatment (300 nM, 1 day) resulted in decreased levels of Ku70 and Ku86 proteins that play a role in DNA repair processes, suggesting that DNA repair processes may have been disrupted by this agent [13].

Physical interactions of Xrcc6


Other interactions of Xrcc6


Analytical, diagnostic and therapeutic context of Xrcc6


  1. Genomic structure and chromosomal assignment of the mouse Ku70 gene. Takiguchi, Y., Kurimasa, A., Chen, F., Pardington, P.E., Kuriyama, T., Okinaka, R.T., Moyzis, R., Chen, D.J. Genomics (1996) [Pubmed]
  2. Growth retardation and leaky SCID phenotype of Ku70-deficient mice. Gu, Y., Seidl, K.J., Rathbun, G.A., Zhu, C., Manis, J.P., van der Stoep, N., Davidson, L., Cheng, H.L., Sekiguchi, J.M., Frank, K., Stanhope-Baker, P., Schlissel, M.S., Roth, D.B., Alt, F.W. Immunity (1997) [Pubmed]
  3. Ku70 is required for DNA repair but not for T cell antigen receptor gene recombination In vivo. Ouyang, H., Nussenzweig, A., Kurimasa, A., Soares, V.C., Li, X., Cordon-Cardo, C., Li, W., Cheong, N., Nussenzweig, M., Iliakis, G., Chen, D.J., Li, G.C. J. Exp. Med. (1997) [Pubmed]
  4. A targeted DNA-PKcs-null mutation reveals DNA-PK-independent functions for KU in V(D)J recombination. Gao, Y., Chaudhuri, J., Zhu, C., Davidson, L., Weaver, D.T., Alt, F.W. Immunity (1998) [Pubmed]
  5. The nonhomologous end-joining pathway of DNA repair is required for genomic stability and the suppression of translocations. Ferguson, D.O., Sekiguchi, J.M., Chang, S., Frank, K.M., Gao, Y., DePinho, R.A., Alt, F.W. Proc. Natl. Acad. Sci. U.S.A. (2000) [Pubmed]
  6. Function of DNA-protein kinase catalytic subunit during the early meiotic prophase without Ku70 and Ku86. Hamer, G., Roepers-Gajadien, H.L., van Duyn-Goedhart, A., Gademan, I.S., Kal, H.B., van Buul, P.P., Ashley, T., de Rooij, D.G. Biol. Reprod. (2003) [Pubmed]
  7. Functional characterization of a novel Ku70/80 pause site at the H19/Igf2 imprinting control region. Katz, D.J., Beer, M.A., Levorse, J.M., Tilghman, S.M. Mol. Cell. Biol. (2005) [Pubmed]
  8. DNA repair factors and telomere-chromosome integrity in mammalian cells. Hande, M.P. Cytogenet. Genome Res. (2004) [Pubmed]
  9. Double strand break rejoining by the Ku-dependent mechanism of non-homologous end-joining. Jeggo, P., Singleton, B., Beamish, H., Priestley, A. C. R. Acad. Sci. III, Sci. Vie (1999) [Pubmed]
  10. Oxidative damage and defective DNA repair is linked to apoptosis of migrating neurons and progenitors during cerebral cortex development in Ku70-deficient mice. Narasimhaiah, R., Tuchman, A., Lin, S.L., Naegele, J.R. Cereb. Cortex (2005) [Pubmed]
  11. Differential etoposide sensitivity of cells deficient in the Ku and DNA-PKcs components of the DNA-dependent protein kinase. Jin, S., Inoue, S., Weaver, D.T. Carcinogenesis (1998) [Pubmed]
  12. Effect of Ku proteins on IRES-mediated translation. Silvera, D., Koloteva-Levine, N., Burma, S., Elroy-Stein, O. Biol. Cell (2006) [Pubmed]
  13. Flavopiridol, a cyclin-dependent kinase inhibitor, enhances radiosensitivity of ovarian carcinoma cells. Raju, U., Nakata, E., Mason, K.A., Ang, K.K., Milas, L. Cancer Res. (2003) [Pubmed]
  14. Role of DNA-dependent protein kinase in neuronal survival. Chechlacz, M., Vemuri, M.C., Naegele, J.R. J. Neurochem. (2001) [Pubmed]
  15. The involvement of key DNA repair pathways in the formation of chromosome rearrangements in embryonic stem cells. Griffin, C., Waard, H., Deans, B., Thacker, J. DNA Repair (Amst.) (2005) [Pubmed]
  16. Ku70 is required for late B cell development and immunoglobulin heavy chain class switching. Manis, J.P., Gu, Y., Lansford, R., Sonoda, E., Ferrini, R., Davidson, L., Rajewsky, K., Alt, F.W. J. Exp. Med. (1998) [Pubmed]
  17. 3,4-Dideoxyglucosone-3-ene induces apoptosis in renal tubular epithelial cells. Justo, P., Sanz, A.B., Egido, J., Ortiz, A. Diabetes (2005) [Pubmed]
  18. Decreased origin usage and initiation of DNA replication in haploinsufficient HCT116 Ku80+/- cells. Sibani, S., Price, G.B., Zannis-Hadjopoulos, M. J. Cell. Sci. (2005) [Pubmed]
  19. Chromosomal localization of the mouse and rat DNA double-strand break repair genes Ku p70 and Ku p80/XRCC5 and their mRNA expression in various mouse tissues. Koike, M., Matsuda, Y., Mimori, T., Harada, Y.N., Shiomi, N., Shiomi, T. Genomics (1996) [Pubmed]
  20. Tissue-specific changes of DNA repair protein Ku and mtHSP70 in aging rats and their retardation by caloric restriction. Um, J.H., Kim, S.J., Kim, D.W., Ha, M.Y., Jang, J.H., Kim, D.W., Chung, B.S., Kang, C.D., Kim, S.H. Mech. Ageing Dev. (2003) [Pubmed]
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