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The RAG2 C-terminus and ATM protect genome integrity by controlling antigen receptor gene cleavage.

Chaumeil J, Micsinai M, Ntziachristos P, Roth DB, Aifantis I, Kluger Y, Deriano L, Skok JA.

Nat Commun. 2013;4:2231. doi: 10.1038/ncomms3231.


The RAG2 C terminus suppresses genomic instability and lymphomagenesis.

Deriano L, Chaumeil J, Coussens M, Multani A, Chou Y, Alekseyenko AV, Chang S, Skok JA, Roth DB.

Nature. 2011 Mar 3;471(7336):119-23. doi: 10.1038/nature09755.


ATM-deficient thymic lymphoma is associated with aberrant tcrd rearrangement and gene amplification.

Zha S, Bassing CH, Sanda T, Brush JW, Patel H, Goff PH, Murphy MM, Tepsuporn S, Gatti RA, Look AT, Alt FW.

J Exp Med. 2010 Jul 5;207(7):1369-80. doi: 10.1084/jem.20100285. Epub 2010 Jun 21.


Higher-order looping and nuclear organization of Tcra facilitate targeted rag cleavage and regulated rearrangement in recombination centers.

Chaumeil J, Micsinai M, Ntziachristos P, Deriano L, Wang JM, Ji Y, Nora EP, Rodesch MJ, Jeddeloh JA, Aifantis I, Kluger Y, Schatz DG, Skok JA.

Cell Rep. 2013 Feb 21;3(2):359-70. doi: 10.1016/j.celrep.2013.01.024. Epub 2013 Feb 14.


ATM deficiency impairs thymocyte maturation because of defective resolution of T cell receptor alpha locus coding end breaks.

Vacchio MS, Olaru A, Livak F, Hodes RJ.

Proc Natl Acad Sci U S A. 2007 Apr 10;104(15):6323-8. Epub 2007 Apr 3.


The Conserved ATM Kinase RAG2-S365 Phosphorylation Site Limits Cleavage Events in Individual Cells Independent of Any Repair Defect.

Hewitt SL, Wong JB, Lee JH, Nishana M, Chen H, Coussens M, Arnal SM, Blumenberg LM, Roth DB, Paull TT, Skok JA.

Cell Rep. 2017 Oct 24;21(4):979-993. doi: 10.1016/j.celrep.2017.09.084.


ATM influences the efficiency of TCRβ rearrangement, subsequent TCRβ-dependent T cell development, and generation of the pre-selection TCRβ CDR3 repertoire.

Hathcock KS, Bowen S, Livak F, Hodes RJ.

PLoS One. 2013 Apr 23;8(4):e62188. doi: 10.1371/journal.pone.0062188. Print 2013.


Abnormal rearrangement within the alpha/delta T-cell receptor locus in lymphomas from Atm-deficient mice.

Liyanage M, Weaver Z, Barlow C, Coleman A, Pankratz DG, Anderson S, Wynshaw-Boris A, Ried T.

Blood. 2000 Sep 1;96(5):1940-6.


The ataxia telangiectasia mutated and cyclin D3 proteins cooperate to help enforce TCRβ and IgH allelic exclusion.

Steinel NC, Fisher MR, Yang-Iott KS, Bassing CH.

J Immunol. 2014 Sep 15;193(6):2881-90. doi: 10.4049/jimmunol.1302201. Epub 2014 Aug 15.


Chimeric IgH-TCRalpha/delta translocations in T lymphocytes mediated by RAG.

Callén E, Bunting S, Huang CY, Difilippantonio MJ, Wong N, Khor B, Mahowald G, Kruhlak MJ, Ried T, Sleckman BP, Nussenzweig A.

Cell Cycle. 2009 Aug;8(15):2408-12. Epub 2009 Aug 21.


ATM and p53 are essential in the cell-cycle containment of DNA breaks during V(D)J recombination in vivo.

Dujka ME, Puebla-Osorio N, Tavana O, Sang M, Zhu C.

Oncogene. 2010 Feb 18;29(7):957-65. doi: 10.1038/onc.2009.394. Epub 2009 Nov 16.


Process for immune defect and chromosomal translocation during early thymocyte development lacking ATM.

Isoda T, Takagi M, Piao J, Nakagama S, Sato M, Masuda K, Ikawa T, Azuma M, Morio T, Kawamoto H, Mizutani S.

Blood. 2012 Jul 26;120(4):789-99. doi: 10.1182/blood-2012-02-413195. Epub 2012 Jun 18.


Redundant and nonredundant functions of ATM and H2AX in αβ T-lineage lymphocytes.

Yin B, Lee BS, Yang-Iott KS, Sleckman BP, Bassing CH.

J Immunol. 2012 Aug 1;189(3):1372-9. doi: 10.4049/jimmunol.1200829. Epub 2012 Jun 22.


Tcrδ translocations that delete the Bcl11b haploinsufficient tumor suppressor gene promote atm-deficient T cell acute lymphoblastic leukemia.

Ehrlich LA, Yang-Iott K, Bassing CH.

Cell Cycle. 2014;13(19):3076-82. doi: 10.4161/15384101.2014.949144.


Aberrant TCRδ rearrangement underlies the T-cell lymphocytopenia and t(12;14) translocation associated with ATM deficiency.

Jiang W, Lee BJ, Li C, Dubois RL, Gostissa M, Alt FW, Zha S.

Blood. 2015 Apr 23;125(17):2665-8. doi: 10.1182/blood-2015-01-622621. Epub 2015 Feb 26.


The ataxia telangiectasia mutated kinase controls Igκ allelic exclusion by inhibiting secondary Vκ-to-Jκ rearrangements.

Steinel NC, Lee BS, Tubbs AT, Bednarski JJ, Schulte E, Yang-Iott KS, Schatz DG, Sleckman BP, Bassing CH.

J Exp Med. 2013 Feb 11;210(2):233-9. doi: 10.1084/jem.20121605. Epub 2013 Feb 4.


Enforcing order within a complex locus: current perspectives on the control of V(D)J recombination at the murine T-cell receptor alpha/delta locus.

Krangel MS, Carabana J, Abbarategui I, Schlimgen R, Hawwari A.

Immunol Rev. 2004 Aug;200:224-32. Review.


ATM deficiency disrupts Tcra locus integrity and the maturation of CD4+CD8+ thymocytes.

Matei IR, Gladdy RA, Nutter LM, Canty A, Guidos CJ, Danska JS.

Blood. 2007 Mar 1;109(5):1887-96. Epub 2006 Oct 31.


RAG-mediated V(D)J recombination is not essential for tumorigenesis in Atm-deficient mice.

Petiniot LK, Weaver Z, Vacchio M, Shen R, Wangsa D, Barlow C, Eckhaus M, Steinberg SM, Wynshaw-Boris A, Ried T, Hodes RJ.

Mol Cell Biol. 2002 May;22(9):3174-7.


Concurrent V(D)J recombination and DNA end instability increase interchromosomal trans-rearrangements in ATM-deficient thymocytes.

Bowen S, Wangsa D, Ried T, Livak F, Hodes RJ.

Nucleic Acids Res. 2013 Apr;41(8):4535-48. doi: 10.1093/nar/gkt154. Epub 2013 Mar 6.

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