D. Buck, D. Moshous, R. De-chasseval, Y. Ma, F. Le-deist et al., Severe combined immunodeficiency and microcephaly in siblings with hypomorphic mutations in DNA ligase IV, European Journal of Immunology, vol.388, issue.1, pp.224-235, 2006.
DOI : 10.1002/eji.200535401

P. Revy, L. Malivert, and J. De-villartay, Cernunnos-XLF, a recently identified non-homologous end-joining factor required for the development of the immune system, Current Opinion in Allergy and Clinical Immunology, vol.6, issue.6, pp.416-420, 2006.
DOI : 10.1097/01.all.0000246623.72365.43

P. Ahnesorg, P. Smith, and S. Jackson, XLF Interacts with the XRCC4-DNA Ligase IV Complex to Promote DNA Nonhomologous End-Joining, Cell, vol.124, issue.2, pp.301-313, 2006.
DOI : 10.1016/j.cell.2005.12.031

S. Andres, M. Modesti, C. Tsai, G. Chu, and M. Junop, Crystal Structure of Human XLF: A Twist in Nonhomologous DNA End-Joining, Molecular Cell, vol.28, issue.6, 2007.
DOI : 10.1016/j.molcel.2007.10.024

Y. Li, D. Chirgadze, V. Bolanos-garcia, B. Sibanda, O. Davies et al., Crystal structure of human XLF/Cernunnos reveals unexpected differences from XRCC4 with implications for NHEJ, The EMBO Journal, vol.40, issue.1, pp.290-300, 2008.
DOI : 10.1038/sj.emboj.7601942

S. Andres, A. Vergnes, D. Ristic, C. Wyman, M. Modesti et al., A human XRCC4-XLF complex bridges DNA, Nucleic Acids Research, vol.40, issue.4, pp.1868-1878, 2012.
DOI : 10.1093/nar/gks022
URL : http://doi.org/10.1093/nar/gks022

M. Hammel, M. Rey, Y. Yu, R. Mani, S. Classen et al., XRCC4 Protein Interactions with XRCC4-like Factor (XLF) Create an Extended Grooved Scaffold for DNA Ligation and Double Strand Break Repair, Journal of Biological Chemistry, vol.286, issue.37, pp.32638-32650, 2011.
DOI : 10.1074/jbc.M111.272641

V. Ropars, P. Drevet, P. Legrand, S. Baconnais, J. Amram et al., Structural characterization of filaments formed by human Xrcc4-Cernunnos/XLF complex involved in nonhomologous DNA end-joining, Proceedings of the National Academy of Sciences, vol.108, issue.31, pp.12663-12668, 2011.
DOI : 10.1073/pnas.1100758108
URL : https://hal.archives-ouvertes.fr/hal-00613194

Q. Wu, T. Ochi, D. Matak-vinkovic, C. Robinson, D. Chirgadze et al., Non-homologous end-joining partners in a helical dance: structural studies of XLF???XRCC4 interactions, Biochemical Society Transactions, vol.37, issue.5, pp.1387-1392, 2011.
DOI : 10.1107/S0021889897006766

C. Tsai, S. Kim, and G. Chu, Cernunnos/XLF promotes the ligation of mismatched and noncohesive DNA ends, Proceedings of the National Academy of Sciences, vol.104, issue.19, pp.7851-7856, 2007.
DOI : 10.1073/pnas.0702620104

K. Akopiants, R. Zhou, S. Mohapatra, K. Valerie, S. Lees-miller et al., Requirement for XLF/Cernunnos in alignment-based gap filling by DNA polymerases ?? and ?? for nonhomologous end joining in human whole-cell extracts, Nucleic Acids Research, vol.37, issue.12, pp.4055-4062, 2009.
DOI : 10.1093/nar/gkp283

J. Gu, H. Lu, A. Tsai, K. Schwarz, and M. Lieber, Single-stranded DNA ligation and XLF-stimulated incompatible DNA end ligation by the XRCC4-DNA ligase IV complex: influence of terminal DNA sequence, Nucleic Acids Research, vol.35, issue.17, pp.5755-5762, 2007.
DOI : 10.1093/nar/gkm579

J. Gu, H. Lu, B. Tippin, N. Shimazaki, M. Goodman et al., XRCC4:DNA ligase IV can ligate incompatible DNA ends and can ligate across gaps, The EMBO Journal, vol.20, issue.4, pp.1010-1023, 2007.
DOI : 10.1038/sj.emboj.7601559
URL : http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1933392

E. Riballo, L. Woodbine, T. Stiff, S. Walker, A. Goodarzi et al., XLF-Cernunnos promotes DNA ligase IV-XRCC4 re-adenylation following ligation, Nucleic Acids Research, vol.37, issue.2, pp.482-492, 2009.
DOI : 10.1093/nar/gkn957
URL : http://doi.org/10.1093/nar/gkn957

L. Woodbine, A. Gennery, and P. Jeggo, The clinical impact of deficiency in DNA non-homologous end-joining, DNA Repair, vol.16, pp.84-96, 2014.
DOI : 10.1016/j.dnarep.2014.02.011

M. Schwartz, Y. Oren, A. Bester, A. Rahat, R. Sfez et al., Impaired Replication Stress Response in Cells from Immunodeficiency Patients Carrying Cernunnos/XLF Mutations, PLoS ONE, vol.277, issue.2, 2009.
DOI : 10.1371/journal.pone.0004516.g006

S. Zha, F. Alt, H. Cheng, J. Brush, and G. Li, Defective DNA repair and increased genomic instability in Cernunnos-XLF-deficient murine ES cells, Proceedings of the National Academy of Sciences, vol.104, issue.11, pp.4518-4523, 2007.
DOI : 10.1073/pnas.0611734104
URL : http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1838633

G. Li, F. Alt, H. Cheng, J. Brush, P. Goff et al., Lymphocyte-Specific Compensation for XLF/Cernunnos End-Joining Functions in V(D)J Recombination, Molecular Cell, vol.31, issue.5, pp.631-640, 2008.
DOI : 10.1016/j.molcel.2008.07.017

S. Avagyan, M. Churchill, K. Yamamoto, J. Crowe, C. Li et al., Hematopoietic stem cell dysfunction underlies the progressive lymphocytopenia in XLF/Cernunnos deficiency, Blood, vol.124, issue.10, pp.1622-1625, 2014.
DOI : 10.1182/blood-2014-05-574863

S. Zha, C. Guo, C. Boboila, V. Oksenych, H. Cheng et al., ATM damage response and XLF repair factor are functionally redundant in joining DNA breaks Functional redundancy between the XLF and DNA-PKcs DNA repair factors in V(D)J recombination and nonhomologous DNA end joining, Nature Proc Natl Acad Sci, vol.469, issue.110, pp.250-2542234, 2011.

V. Oksenych, F. Alt, V. Kumar, B. Schwer, D. Wesemann et al., Functional redundancy between repair factor XLF and damage response mediator 53BP1 in V(D)J recombination and DNA repair, Proceedings of the National Academy of Sciences, vol.109, issue.7, pp.2455-2460, 2012.
DOI : 10.1073/pnas.1121458109

S. Zha, J. Sekiguchi, J. Brush, C. Bassing, and F. Alt, Complementary functions of ATM and H2AX in development and suppression of genomic instability, Proceedings of the National Academy of Sciences, vol.105, issue.27, pp.9302-9306, 2008.
DOI : 10.1073/pnas.0803520105

X. Liu, W. Jiang, R. Dubois, K. Yamamoto, Z. Wolner et al., Overlapping functions between XLF repair protein and 53BP1 DNA damage response factor in end joining and lymphocyte development, Proceedings of the National Academy of Sciences, vol.109, issue.10, pp.3903-3908, 2012.
DOI : 10.1073/pnas.1120160109
URL : http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3309750

M. Xing, M. Yang, W. Huo, F. Feng, L. Wei et al., Interactome analysis identifies a new paralogue of XRCC4 in non-homologous end joining DNA repair pathway, Nature Communications, vol.6, p.6233, 2015.
DOI : 10.1038/emboj.2012.304

T. Ochi, A. Blackford, J. Coates, S. Jhujh, S. Mehmood et al., PAXX, a paralog of XRCC4 and XLF, interacts with Ku to promote DNA double-strand break repair, Science, vol.347, issue.6218, pp.185-188, 2015.
DOI : 10.1126/science.1261971

C. Lundin, K. Erixon, C. Arnaudeau, N. Schultz, D. Jenssen et al., Different Roles for Nonhomologous End Joining and Homologous Recombination following Replication Arrest in Mammalian Cells, Molecular and Cellular Biology, vol.22, issue.16, pp.5869-5878, 2002.
DOI : 10.1128/MCB.22.16.5869-5878.2002

Y. Saintigny, F. Delacote, G. Vares, F. Petitot, S. Lambert et al., Characterization of homologous recombination induced by replication inhibition in mammalian cells, The EMBO Journal, vol.20, issue.14, pp.3861-3870, 2001.
DOI : 10.1093/emboj/20.14.3861

S. Roy, S. Andres, A. Vergnes, J. Neal, Y. Xu et al., XRCC4's interaction with XLF is required for coding (but not signal) end joining, Nucleic Acids Research, vol.40, issue.4, pp.1684-1694, 2012.
DOI : 10.1093/nar/gkr1315
URL : http://doi.org/10.1093/nar/gkr1315

J. Neal, V. Dang, P. Douglas, M. Wold, S. Lees-miller et al., Inhibition of Homologous Recombination by DNA-Dependent Protein Kinase Requires Kinase Activity, Is Titratable, and Is Modulated by Autophosphorylation, Molecular and Cellular Biology, vol.31, issue.8, pp.1719-1733, 2011.
DOI : 10.1128/MCB.01298-10

M. Junop, M. Modesti, A. Guarne, R. Ghirlando, M. Gellert et al., Crystal structure of the Xrcc4 DNA repair protein and implications for end joining, The EMBO Journal, vol.19, issue.22, pp.5962-5970, 2000.
DOI : 10.1093/emboj/19.22.5962

J. Cottarel, P. Frit, O. Bombarde, B. Salles, A. Negrel et al., A noncatalytic function of the ligation complex during nonhomologous end joining, The Journal of Cell Biology, vol.15, issue.2, pp.173-186, 2013.
DOI : 10.1038/nsmb1261

M. Ono, P. Tucker, and J. Capra, Production and characterization of recombinant human Ku antigen, Nucleic Acids Research, vol.22, issue.19, pp.3918-3924, 1994.
DOI : 10.1093/nar/22.19.3918

P. Mali, L. Yang, K. Esvelt, J. Aach, M. Guell et al., RNA-Guided Human Genome Engineering via Cas9, Science, vol.339, issue.6121, pp.823-826, 2013.
DOI : 10.1126/science.1232033
URL : http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3712628

J. Hesse, M. Lieber, M. Gellert, and K. Mizuuchi, Extrachromosomal DNA substrates in pre-B cells undergo inversion or deletion at immunoglobulin V-(D)-J joining signals, Cell, vol.49, issue.6, pp.775-7830092, 1987.
DOI : 10.1016/0092-8674(87)90615-5

C. Piechaczek, C. Fetzer, A. Baiker, J. Bode, and H. Lipps, A vector based on the SV40 origin of replication and chromosomal S/MARs replicates episomally in CHO cells, Nucleic Acids Research, vol.27, issue.2, pp.426-428, 1999.
DOI : 10.1093/nar/27.2.426

L. Malivert, V. Ropars, M. Nunez, P. Drevet, S. Miron et al., Delineation of the Xrcc4-interacting Region in the Globular Head Domain of Cernunnos/XLF, Journal of Biological Chemistry, vol.285, issue.34, pp.26475-26483, 2010.
DOI : 10.1074/jbc.M110.138156
URL : https://hal.archives-ouvertes.fr/hal-00515366

F. Fattah, J. Kweon, Y. Wang, E. Lee, Y. Kan et al., A role for XLF in DNA repair and recombination in human somatic cells, DNA Repair, vol.15, pp.39-53, 2014.
DOI : 10.1016/j.dnarep.2013.12.006

Y. Dai, B. Kysela, L. Hanakahi, K. Manolis, E. Riballo et al., Nonhomologous end joining and V(D)J recombination require an additional factor, Proceedings of the National Academy of Sciences, vol.100, issue.5, pp.2462-2467, 2003.
DOI : 10.1073/pnas.0437964100
URL : http://www.ncbi.nlm.nih.gov/pmc/articles/PMC151363

M. Koi, A. Umar, D. Chauhan, S. Cherian, J. Carethers et al., Human chromosome 3 corrects mismatch repair deficiency and microsatellite instability and reduces N-methyl-N=-nitro-Nnitrosoguanidine tolerance in colon tumor cells with homozygous hMLH1 mutation, Cancer Res, vol.54, pp.4308-4312, 1994.

Q. Wen, J. Scorah, G. Phear, G. Rodgers, S. Rodgers et al., A Mutant Allele of MRE11 Found in Mismatch Repair-deficient Tumor Cells Suppresses the Cellular Response to DNA Replication Fork Stress in a Dominant Negative Manner, Molecular Biology of the Cell, vol.19, issue.4, pp.1693-1705, 2008.
DOI : 10.1091/mbc.E07-09-0975

L. Malivert, I. Callebaut, P. Rivera-munoz, A. Fischer, J. Mornon et al., The C-Terminal Domain of Cernunnos/XLF Is Dispensable for DNA Repair In Vivo, Molecular and Cellular Biology, vol.29, issue.5, pp.1116-1122, 2009.
DOI : 10.1128/MCB.01521-08
URL : https://hal.archives-ouvertes.fr/hal-00361521

M. Lieber, J. Hesse, S. Lewis, G. Bosma, N. Rosenberg et al., The defect in murine severe combined immune deficiency: Joining of signal sequences but not coding segments in V(D)J recombination, Cell, vol.55, issue.1, pp.7-160092, 1988.
DOI : 10.1016/0092-8674(88)90004-9

R. Roy, B. Meier, A. Mcainsh, H. Feldmann, and S. Jackson, Separation-of-function Mutants of Yeast Ku80 Reveal a Yku80p-Sir4p Interaction Involved in Telomeric Silencing, Journal of Biological Chemistry, vol.279, issue.1, pp.86-94, 2004.
DOI : 10.1074/jbc.M306841200

N. Bennardo and J. Stark, ATM Limits Incorrect End Utilization during Non-Homologous End Joining of Multiple Chromosome Breaks, PLoS Genetics, vol.7, issue.11, 2010.
DOI : 10.1371/journal.pgen.1001194.s003

C. Waters, N. Strande, J. Pryor, C. Strom, P. Mieczkowski et al., The fidelity of the ligation step determines how ends are resolved during nonhomologous end joining, Nature Communications, vol.283, p.4286, 2014.
DOI : 10.2307/2347977

Y. Ma and M. Lieber, In Vitro Nonhomologous DNA End Joining System, Methods Enzymol, vol.408, pp.502-510, 2006.
DOI : 10.1016/S0076-6879(06)08031-1

R. Scully and A. Xie, Double strand break repair functions of histone H2AX, Mutation Research/Fundamental and Molecular Mechanisms of Mutagenesis, vol.750, issue.1-2, pp.5-14, 2013.
DOI : 10.1016/j.mrfmmm.2013.07.007

D. Roth, J. Menetski, P. Nakajima, M. Bosma, and M. Gellert, V(D)J recombination: Broken DNA molecules with covalently sealed (hairpin) coding ends in scid mouse thymocytes, Cell, vol.70, issue.6, pp.983-9910092, 1992.
DOI : 10.1016/0092-8674(92)90248-B

Y. Ma, U. Pannicke, K. Schwarz, and M. Lieber, Hairpin Opening and Overhang Processing by an Artemis/DNA-Dependent Protein Kinase Complex in Nonhomologous End Joining and V(D)J Recombination, Cell, vol.108, issue.6, pp.781-794, 2002.
DOI : 10.1016/S0092-8674(02)00671-2

R. Gerstein and M. Lieber, Coding end sequence can markedly affect the initiation of V(D)J recombination., Genes & Development, vol.7, issue.7b, pp.1459-1469, 1993.
DOI : 10.1101/gad.7.7b.1459

K. Meek, P. Douglas, X. Cui, Q. Ding, and S. Lees-miller, trans Autophosphorylation at DNA-Dependent Protein Kinase's Two Major Autophosphorylation Site Clusters Facilitates End Processing but Not End Joining, Molecular and Cellular Biology, vol.27, issue.10, pp.3881-389002366, 2007.
DOI : 10.1128/MCB.02366-06
URL : http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1899996

J. Neal, S. Sugiman-marangos, P. Vandervere-carozza, M. Wagner, J. Turchi et al., Unraveling the Complexities of DNA-Dependent Protein Kinase Autophosphorylation, Molecular and Cellular Biology, vol.34, issue.12, pp.2162-217501554, 2014.
DOI : 10.1128/MCB.01554-13

Y. Ma, H. Lu, K. Schwarz, and M. Lieber, Repair of Double-Strand DNA Breaks by the Human Nonhomologous DNA End Joining Pathway: The Iterative Processing Model, Cell Cycle, vol.4, issue.9, pp.1193-1200, 1977.
DOI : 10.4161/cc.4.9.1977

S. Yoo and W. Dynan, Geometry of a complex formed by double strand break repair proteins at a single DNA end: recruitment of DNA-PKcs induces inward translocation of Ku protein, Nucleic Acids Research, vol.27, issue.24, pp.4679-4686, 1999.
DOI : 10.1093/nar/27.24.4679

D. Reid, S. Keegan, A. Leo-macias, G. Watanabe, N. Strande et al., Organization and dynamics of the nonhomologous end-joining machinery during DNA double-strand break repair, Proceedings of the National Academy of Sciences, vol.112, issue.20, pp.2575-2584, 2015.
DOI : 10.1073/pnas.1420115112

L. Defazio, R. Stansel, J. Griffith, and G. Chu, Synapsis of DNA ends by DNA-dependent protein kinase, The EMBO Journal, vol.21, issue.12, pp.3192-3200, 2002.
DOI : 10.1093/emboj/cdf299

E. Weterings, N. Verkaik, H. Bruggenwirth, J. Hoeijmakers, and D. Van-gent, The role of DNA dependent protein kinase in synapsis of DNA ends, Nucleic Acids Research, vol.31, issue.24, pp.7238-7246, 2003.
DOI : 10.1093/nar/gkg889

G. Grundy, H. Moulding, K. Caldecott, and S. Rulten, One ring to bring them all???The role of Ku in mammalian non-homologous end joining, DNA Repair, vol.17, pp.30-38, 2014.
DOI : 10.1016/j.dnarep.2014.02.019

J. Walker, R. Corpina, and J. Goldberg, Structure of the Ku heterodimer bound to DNA and its implications for double-strand break repair, Nature, vol.412, issue.6847, pp.607-614, 2001.
DOI : 10.1038/35088000

L. Postow, Destroying the ring: Freeing DNA from Ku with ubiquitin, FEBS Letters, vol.71, issue.18, pp.2876-2882, 2011.
DOI : 10.1016/j.febslet.2011.05.046
URL : http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3172340

A. Rivera-calzada, J. Maman, L. Spagnolo, L. Pearl, and O. Llorca, Three-Dimensional Structure and Regulation of the DNA-Dependent Protein Kinase Catalytic Subunit (DNA-PKcs), Structure, vol.13, issue.2, pp.243-255, 2005.
DOI : 10.1016/j.str.2004.12.006

K. Yano, K. Morotomi-yano, S. Wang, N. Uematsu, K. Lee et al., Ku recruits XLF to DNA double-strand breaks, EMBO reports, vol.13, issue.1, pp.91-96, 2008.
DOI : 10.1074/jbc.274.28.20034
URL : http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2246615

K. Yano, K. Morotomi-yano, K. Lee, and D. Chen, Functional significance of the interaction with Ku in DNA double-strand break recognition of XLF, FEBS Letters, vol.6, issue.6, pp.841-846, 2011.
DOI : 10.1016/j.febslet.2011.02.020

M. Lieber, The Mechanism of Human Nonhomologous DNA End Joining, Journal of Biological Chemistry, vol.283, issue.1, pp.1-5, 2008.
DOI : 10.1074/jbc.R700039200

Y. Ma, H. Lu, B. Tippin, M. Goodman, N. Shimazaki et al., A Biochemically Defined System for Mammalian Nonhomologous DNA End Joining, Molecular Cell, vol.16, issue.5, pp.701-713, 2004.
DOI : 10.1016/j.molcel.2004.11.017

M. Lieber, The Mechanism of Double-Strand DNA Break Repair by the Nonhomologous DNA End-Joining Pathway, Annual Review of Biochemistry, vol.79, issue.1, pp.181-211, 2010.
DOI : 10.1146/annurev.biochem.052308.093131

E. Weterings, N. Verkaik, G. Keijzers, B. Florea, S. Wang et al., The Ku80 Carboxy Terminus Stimulates Joining and Artemis-Mediated Processing of DNA Ends, Molecular and Cellular Biology, vol.29, issue.5, pp.1134-114200971, 2009.
DOI : 10.1128/MCB.00971-08

K. Meek, S. Gupta, D. Ramsden, and S. Lees-miller, The DNA-dependent protein kinase: the director at the end, Immunological Reviews, vol.169, issue.1, pp.132-141, 2004.
DOI : 10.1093/emboj/21.11.2827

S. Oh, A. Harvey, J. Zimbric, Y. Wang, T. Nguyen et al., DNA ligase III and DNA ligase IV carry out genetically distinct forms of end joining in human somatic cells, DNA Repair, vol.21, pp.97-110, 2014.
DOI : 10.1016/j.dnarep.2014.04.015

M. Lieber, H. Lu, J. Gu, and K. Schwarz, Flexibility in the order of action and in the enzymology of the nuclease, polymerases, and ligase of vertebrate non-homologous DNA end joining: relevance to cancer, aging, and the immune system, Cell Research, vol.56, issue.1, pp.125-133, 2007.
DOI : 10.1016/j.dnarep.2006.05.030