NBN / Nibrin

Component of the MRE11-RAD50-NBN (MRN complex) which plays a critical role in the cellular response to DNA damage and the maintenance of chromosome integrity. The complex is involved in double-strand break (DSB) repair, DNA recombination, maintenance of telomere integrity, cell cycle checkpoint control and meiosis. The complex possesses single-strand endonuclease activity and double-strand-specific 3'-5' exonuclease activity, which are provided by MRE11A. RAD50 may be required to bind DNA ends and hold them in close proximity. NBN modulate the DNA damage signal sensing by recruiting PI3/PI4-kinase family members ATM, ATR, and probably DNA-PKcs to the DNA damage sites and activating their functions. It can also recruit MRE11 and RAD50 to the proximity of DSBs by an interaction with the histone H2AX. NBN also functions in telomere length maintenance by generating the 3' overhang which serves as a primer for telomerase dependent telomere elongation. NBN is a major player in the control of intra-S-phase checkpoint and there is some evidence that NBN is involved in G1 and G2 checkpoints. The roles of NBS1/MRN encompass DNA damage sensor, signal transducer, and effector, which enable cells to maintain DNA integrity and genomic stability. Forms a complex with RBBP8 to link DNA double-strand break sensing to resection. Enhances AKT1 phosphorylation possibly by association with the mTORC2 complex.

Publications (95)

BASC, a super complex of BRCA1-associated proteins involved in the recognition and repair of aberrant DNA structures. Wang Y, Cortez D, Yazdi P, Neff N, Elledge SJ, Qin J. Genes & development. 2000 14:927-39. (ICC, IHC, WB, IP; Human) [PubMed:10783165] [PMC:PMC316544]

ATM-dependent phosphorylation of nibrin in response to radiation exposure. Gatei M, Young D, Cerosaletti KM, Desai-Mehta A, Spring K, Kozlov S, Lavin MF, Gatti RA, Concannon P, Khanna K. Nature genetics. 2000 25:115-9. [PubMed:10802669]

NBS1 and TRF1 colocalize at promyelocytic leukemia bodies during late S/G2 phases in immortalized telomerase-negative cells. Implication of NBS1 in alternative lengthening of telomeres. Wu G, Lee WH, Chen PL. The Journal of biological chemistry. 2000 275:30618-22. (IP; Human) [PubMed:10913111]

Independence of R/M/N focus formation and the presence of intact BRCA1. Wu X, Petrini JH, Heine WF, Weaver DT, Livingston DM, Chen J. Science (New York, N.Y.). 2000 289:11. [PubMed:10928918]

A critical role for histone H2AX in recruitment of repair factors to nuclear foci after DNA damage. Paull TT, Rogakou EP, Yamazaki V, Kirchgessner CU, Gellert M, Bonner WM. Current biology : CB. 10:886-95. (ICC; Human) [PubMed:10959836]

Chk2 activation dependence on Nbs1 after DNA damage. Buscemi G, Savio C, Zannini L, Miccich F, Masnada D, Nakanishi M, Tauchi H, Komatsu K, Mizutani S, Khanna K, Chen P, Concannon P, Chessa L, Delia D. Molecular and cellular biology. 2001 21:5214-22. (ICC, WB, IP; Human) [PubMed:11438675] [PMC:PMC87245]

Reconstitution of the mammalian DNA double-strand break end-joining reaction reveals a requirement for an Mre11/Rad50/NBS1-containing fraction. Huang J, Dynan WS. Nucleic acids research. 2002 30:667-74. [PubMed:11809878] [PMC:PMC100305]

Human MRE11 is inactivated in mismatch repair-deficient cancers. Giannini G, Ristori E, Cerignoli F, Rinaldi C, Zani M, Viel A, Ottini L, Crescenzi M, Martinotti S, Bignami M, Frati L, Screpanti I, Gulino A. EMBO reports. 2002 3:248-54. [PubMed:11850399] [PMC:PMC1084012]

Bloom's syndrome protein is required for correct relocalization of RAD50/MRE11/NBS1 complex after replication fork arrest. Franchitto A, Pichierri P. The Journal of cell biology. 2002 157:19-30. (ICC, WB, IP; Human) [PubMed:11916980] [PMC:PMC2173275]

Functional link between BLM defective in Bloom's syndrome and the ataxia-telangiectasia-mutated protein, ATM. Beamish H, Kedar P, Kaneko H, Chen P, Fukao T, Peng C, Beresten S, Gueven N, Purdie D, Lees-Miller S, Ellis N, Kondo N, Lavin MF. The Journal of biological chemistry. 2002 277:30515-23. (WB, IP, Flo; Human) [PubMed:12034743]

Adenovirus oncoproteins inactivate the Mre11-Rad50-NBS1 DNA repair complex. Stracker TH, Carson CT, Weitzman MD. Nature. 2002 418:348-52. [PubMed:12124628]

The mismatch repair system is required for S-phase checkpoint activation. Brown KD, Rathi A, Kamath R, Beardsley DI, Zhan Q, Mannino JL, Baskaran R. Nature genetics. 2003 33:80-4. [PubMed:12447371]

Distinct spatiotemporal dynamics of mammalian checkpoint regulators induced by DNA damage. Lukas C, Falck J, Bartkova J, Bartek J, Lukas J. Nature cell biology. 2003 5:255-60. [PubMed:12598907]

MDC1 is required for the intra-S-phase DNA damage checkpoint. Goldberg M, Stucki M, Falck J, D'Amours D, Rahman D, Pappin D, Bartek J, Jackson SP. Nature. 2003 421:952-6. (ICC; Human) [PubMed:12607003]

Nibrin forkhead-associated domain and breast cancer C-terminal domain are both required for nuclear focus formation and phosphorylation. Cerosaletti KM, Concannon P. The Journal of biological chemistry. 2003 278:21944-51. (ICC, WB, IP; Human) [PubMed:12679336]

Deficient regulation of DNA double-strand break repair in Fanconi anemia fibroblasts. Donahue SL, Lundberg R, Saplis R, Campbell C. The Journal of biological chemistry. 2003 278:29487-95. (WB; Human) [PubMed:12748186]

Telomere maintenance and cell cycle regulation in spontaneously immortalized T-cell lines from Nijmegen breakage syndrome patients. Siwicki JK, Degerman S, Chrzanowska KH, Roos G. Experimental cell research. 2003 287:178-89. (WB, IP, Flo; Human) [PubMed:12799193]

Distinct functions of Nijmegen breakage syndrome in ataxia telangiectasia mutated-dependent responses to DNA damage. Lee JH, Xu B, Lee CH, Ahn JY, Song MS, Lee H, Canman CE, Lee JS, Kastan MB, Lim DS. Molecular cancer research : MCR. 2003 1:674-81. (WB, IP; Human) [PubMed:12861053]

Recapitulation of the cellular xeroderma pigmentosum-variant phenotypes using short interfering RNA for DNA polymerase H. Laposa RR, Feeney L, Cleaver JE. Cancer research. 2003 63:3909-12. (WB; Human) [PubMed:12873983]

Distinct pathways of nonhomologous end joining that are differentially regulated by DNA-dependent protein kinase-mediated phosphorylation. Udayakumar D, Bladen CL, Hudson FZ, Dynan WS. The Journal of biological chemistry. 2003 278:41631-5. (WB; Human) [PubMed:12917393]

Suppression of Tousled-like kinase activity after DNA damage or replication block requires ATM, NBS1 and Chk1. Krause DR, Jonnalagadda JC, Gatei MH, Sillje HH, Zhou BB, Nigg EA, Khanna K. Oncogene. 2003 22:5927-37. [PubMed:12955071]

Bloom syndrome cells undergo p53-dependent apoptosis and delayed assembly of BRCA1 and NBS1 repair complexes at stalled replication forks. Davalos AR, Campisi J. The Journal of cell biology. 2003 162:1197-209. (ICC, WB, IP; Human) [PubMed:14517203] [PMC:PMC2173967]

NFBD1/MDC1 regulates ionizing radiation-induced focus formation by DNA checkpoint signaling and repair factors. Xu X, Stern DF. FASEB journal : official publication of the Federation of American Societies for Experimental Biology. 2003 17:1842-8. (ICC; Human) [PubMed:14519663]

Chk2 phosphorylation of BRCA1 regulates DNA double-strand break repair. Zhang J, Willers H, Feng Z, Ghosh JC, Kim S, Weaver DT, Chung JH, Powell SN, Xia F. Molecular and cellular biology. 2004 24:708-18. (ICC, WB, IP; Human) [PubMed:14701743] [PMC:PMC343805]

Linkage between Werner syndrome protein and the Mre11 complex via Nbs1. Cheng WH, von Kobbe C, Opresko PL, Arthur LM, Komatsu K, Seidman MM, Carney JP, Bohr VA. The Journal of biological chemistry. 2004 279:21169-76. (ICC, WB, IP; Human) [PubMed:15026416]

Recruitment of cellular recombination and repair proteins to sites of herpes simplex virus type 1 DNA replication is dependent on the composition of viral proteins within prereplicative sites and correlates with the induction of the DNA damage response. Wilkinson DE, Weller SK. Journal of virology. 2004 78:4783-96. (ICC, WB; Primate) [PubMed:15078960] [PMC:PMC387708]

BRCA1-BARD1 complexes are required for p53Ser-15 phosphorylation and a G1/S arrest following ionizing radiation-induced DNA damage. Fabbro M, Savage K, Hobson K, Deans AJ, Powell SN, McArthur GA, Khanna KK. The Journal of biological chemistry. 2004 279:31251-8. (ICC, WB; Human) [PubMed:15159397]

Phosphorylation of SMC1 is a critical downstream event in the ATM-NBS1-BRCA1 pathway. Kitagawa R, Bakkenist CJ, McKinnon PJ, Kastan MB. Genes & development. 2004 18:1423-38. (ICC, WB; Mouse, Human) [PubMed:15175241] [PMC:PMC423193]

A Rad50-dependent pathway of DNA repair is deficient in Fanconi anemia fibroblasts. Donahue SL, Campbell C. Nucleic acids research. 2004 32:3248-57. (Human) [PubMed:15199173] [PMC:PMC434453]

Mdc1 couples DNA double-strand break recognition by Nbs1 with its H2AX-dependent chromatin retention. Lukas C, Melander F, Stucki M, Falck J, Bekker-Jensen S, Goldberg M, Lerenthal Y, Jackson SP, Bartek J, Lukas J. The EMBO journal. 2004 23:2674-83. (Human) [PubMed:15201865] [PMC:PMC449779]

Independent roles for nibrin and Mre11-Rad50 in the activation and function of Atm. Cerosaletti K, Concannon P. The Journal of biological chemistry. 2004 279:38813-9. (ICC, WB; Human) [PubMed:15234984]

An inducible null mutant murine model of Nijmegen breakage syndrome proves the essential function of NBS1 in chromosomal stability and cell viability. Demuth I, Frappart PO, Hildebrand G, Melchers A, Lobitz S, Stckl L, Varon R, Herceg Z, Sperling K, Wang ZQ, Digweed M. Human molecular genetics. 2004 13:2385-97. [PubMed:15333589]

Evasion of early cellular response mechanisms following low level radiation-induced DNA damage. Collis SJ, Schwaninger JM, Ntambi AJ, Keller TW, Nelson WG, Dillehay LE, Deweese TL. The Journal of biological chemistry. 2004 279:49624-32. (ICC, WB, Flo; Human) [PubMed:15377658]

Human SNM1B is required for normal cellular response to both DNA interstrand crosslink-inducing agents and ionizing radiation. Demuth I, Digweed M, Concannon P. Oncogene. 2004 23:8611-8. [PubMed:15467758]

Defective DNA strand break repair after DNA damage in prostate cancer cells: implications for genetic instability and prostate cancer progression. Fan R, Kumaravel TS, Jalali F, Marrano P, Squire JA, Bristow RG. Cancer research. 2004 64:8526-33. [PubMed:15574758]

The Fanconi anemia pathway is required for the DNA replication stress response and for the regulation of common fragile site stability. Howlett NG, Taniguchi T, Durkin SG, D'Andrea AD, Glover TW. Human molecular genetics. 2005 14:693-701. [PubMed:15661754]

Mdm2 binds to Nbs1 at sites of DNA damage and regulates double strand break repair. Alt JR, Bouska A, Fernandez MR, Cerny RL, Xiao H, Eischen CM. The Journal of biological chemistry. 2005 280:18771-81. (ICC, WB; Human) [PubMed:15734743]

DNA repair proteins affect the lifecycle of herpes simplex virus 1. Lilley CE, Carson CT, Muotri AR, Gage FH, Weitzman MD. Proceedings of the National Academy of Sciences of the United States of America. 2005 102:5844-9. (WB; Human) [PubMed:15824307] [PMC:PMC556126]

Farnesyltransferase inhibitors induce DNA damage via reactive oxygen species in human cancer cells. Pan J, She M, Xu ZX, Sun L, Yeung SC. Cancer research. 2005 65:3671-81. [PubMed:15867362]

Nijmegen breakage syndrome (NBS) with neurological abnormalities and without chromosomal instability. Seemanov E, Sperling K, Neitzel H, Varon R, Hadac J, Butova O, Schrck E, Seeman P, Digweed M. Journal of medical genetics. 2006 43:218-24. [PubMed:16033915] [PMC:PMC2563240]

Importin KPNA2 is required for proper nuclear localization and multiple functions of NBS1. Tseng SF, Chang CY, Wu KJ, Teng SC. The Journal of biological chemistry. 2005 280:39594-600. [PubMed:16188882]

Functional human telomeres are recognized as DNA damage in G2 of the cell cycle. Verdun RE, Crabbe L, Haggblom C, Karlseder J. Molecular cell. 2005 20:551-61. [PubMed:16307919]

Mild Nijmegen breakage syndrome phenotype due to alternative splicing. Varon R, Dutrannoy V, Weikert G, Tanzarella C, Antoccia A, Stckl L, Spadoni E, Krger LA, di Masi A, Sperling K, Digweed M, Maraschio P. Human molecular genetics. 2006 15:679-89. [PubMed:16415040]

Increased NBS1 expression is a marker of aggressive head and neck cancer and overexpression of NBS1 contributes to transformation. Yang MH, Chiang WC, Chou TY, Chang SY, Chen PM, Teng SC, Wu KJ. Clinical cancer research : an official journal of the American Association for Cancer Research. 2006 12:507-15. [PubMed:16428493]

Rapid activation of ATR by ionizing radiation requires ATM and Mre11. Myers JS, Cortez D. The Journal of biological chemistry. 2006 281:9346-50. [PubMed:16431910] [PMC:PMC1821075]

Defective p53 response and apoptosis associated with an ataxia-telangiectasia-like phenotype. Gueven N, Becherel OJ, Birrell G, Chen P, DelSal G, Carney JP, Grattan-Smith P, Lavin MF. Cancer research. 2006 66:2907-12. [PubMed:16540636]

Mre11-Rad50-Nbs1 complex is activated by hypertonicity. Sheen MR, Kim SW, Jung JY, Ahn JY, Rhee JG, Kwon HM, Woo SK. American journal of physiology. Renal physiology. 2006 291:F1014-20. [PubMed:16788144]

Cancer incidence in Nijmegen breakage syndrome is modulated by the amount of a variant NBS protein. Krger L, Demuth I, Neitzel H, Varon R, Sperling K, Chrzanowska KH, Seemanova E, Digweed M. Carcinogenesis. 2007 28:107-11. [PubMed:16840438]

Defective Mre11-dependent activation of Chk2 by ataxia telangiectasia mutated in colorectal carcinoma cells in response to replication-dependent DNA double strand breaks. Takemura H, Rao VA, Sordet O, Furuta T, Miao ZH, Meng L, Zhang H, Pommier Y. The Journal of biological chemistry. 2006 281:30814-23. [PubMed:16905549]

Deficiency in the repair of DNA damage by homologous recombination and sensitivity to poly(ADP-ribose) polymerase inhibition. McCabe N, Turner NC, Lord CJ, Kluzek K, Bialkowska A, Swift S, Giavara S, O'Connor MJ, Tutt AN, Zdzienicka MZ, Smith GC, Ashworth A. Cancer research. 2006 66:8109-15. [PubMed:16912188]

Inhibition of hsp90 compromises the DNA damage response to radiation. Dote H, Burgan WE, Camphausen K, Tofilon PJ. Cancer research. 2006 66:9211-20. [PubMed:16982765]

Xrcc3 and Nbs1 are required for the production of extrachromosomal telomeric circles in human alternative lengthening of telomere cells. Compton SA, Choi JH, Cesare AJ, Ozgr S, Griffith JD. Cancer research. 2007 67:1513-9. [PubMed:17308089]

Proteasome function is required for DNA damage response and fanconi anemia pathway activation. Jacquemont C, Taniguchi T. Cancer research. 2007 67:7395-405. (ICC; Human) [PubMed:17671210]

Activation of Holliday junction recognizing protein involved in the chromosomal stability and immortality of cancer cells. Kato T, Sato N, Hayama S, Yamabuki T, Ito T, Miyamoto M, Kondo S, Nakamura Y, Daigo Y. Cancer research. 2007 67:8544-53. [PubMed:17823411]

The Mre11/Rad50/Nbs1 complex limits adeno-associated virus transduction and replication. Schwartz RA, Palacios JA, Cassell GD, Adam S, Giacca M, Weitzman MD. Journal of virology. 2007 81:12936-45. [PubMed:17898048] [PMC:PMC2169118]

RIDDLE immunodeficiency syndrome is linked to defects in 53BP1-mediated DNA damage signaling. Stewart GS, Stankovic T, Byrd PJ, Wechsler T, Miller ES, Huissoon A, Drayson MT, West SC, Elledge SJ, Taylor AM. Proceedings of the National Academy of Sciences of the United States of America. 2007 104:16910-5. [PubMed:17940005] [PMC:PMC2040433]

Resveratrol modulates DNA double-strand break repair pathways in an ATM/ATR-p53- and -Nbs1-dependent manner. Gatz SA, Keimling M, Baumann C, Drk T, Debatin KM, Fulda S, Wiesmller L. Carcinogenesis. 2008 29:519-27. [PubMed:18174244]

Processing of recombinant AAV genomes occurs in specific nuclear structures that overlap with foci of DNA-damage-response proteins. Cervelli T, Palacios JA, Zentilin L, Mano M, Schwartz RA, Weitzman MD, Giacca M. Journal of cell science. 2008 121:349-57. [PubMed:18216333]

A glycine-arginine domain in control of the human MRE11 DNA repair protein. Dry U, Coulombe Y, Rodrigue A, Stasiak A, Richard S, Masson JY. Molecular and cellular biology. 2008 28:3058-69. [PubMed:18285453] [PMC:PMC2293076]

Ataxia telangiectasia-mutated damage-signaling kinase- and proteasome-dependent destruction of Mre11-Rad50-Nbs1 subunits in Simian virus 40-infected primate cells. Zhao X, Madden-Fuentes RJ, Lou BX, Pipas JM, Gerhardt J, Rigell CJ, Fanning E. Journal of virology. 2008 82:5316-28. [PubMed:18353955] [PMC:PMC2395194]

Constitutive phosphorylation of MDC1 physically links the MRE11-RAD50-NBS1 complex to damaged chromatin. Spycher C, Miller ES, Townsend K, Pavic L, Morrice NA, Janscak P, Stewart GS, Stucki M. The Journal of cell biology. 2008 181:227-40. [PubMed:18411308] [PMC:PMC2315671]

Mdm2 promotes genetic instability and transformation independent of p53. Bouska A, Lushnikova T, Plaza S, Eischen CM. Molecular and cellular biology. 2008 28:4862-74. [PubMed:18541670] [PMC:PMC2493369]

Differential requirements of the C terminus of Nbs1 in suppressing adenovirus DNA replication and promoting concatemer formation. Lakdawala SS, Schwartz RA, Ferenchak K, Carson CT, McSharry BP, Wilkinson GW, Weitzman MD. Journal of virology. 2008 82:8362-72. [PubMed:18562516] [PMC:PMC2519655]

Distinct requirements of adenovirus E1b55K protein for degradation of cellular substrates. Schwartz RA, Lakdawala SS, Eshleman HD, Russell MR, Carson CT, Weitzman MD. Journal of virology. 2008 82:9043-55. [PubMed:18614635] [PMC:PMC2546875]

A role for DEAD box 1 at DNA double-strand breaks. Li L, Monckton EA, Godbout R. Molecular and cellular biology. 2008 28:6413-25. [PubMed:18710941] [PMC:PMC2577411]

ATM and the Mre11-Rad50-Nbs1 complex respond to nucleoside analogue-induced stalled replication forks and contribute to drug resistance. Ewald B, Sampath D, Plunkett W. Cancer research. 2008 68:7947-55. [PubMed:18829552] [PMC:PMC2631429]

Role for proteasome activator PA200 and postglutamyl proteasome activity in genomic stability. Blickwedehl J, Agarwal M, Seong C, Pandita RK, Melendy T, Sung P, Pandita TK, Bangia N. Proceedings of the National Academy of Sciences of the United States of America. 2008 105:16165-70. [PubMed:18845680] [PMC:PMC2570974]

Rapid flow cytometry-based structural maintenance of chromosomes 1 (SMC1) phosphorylation assay for identification of ataxia-telangiectasia homozygotes and heterozygotes. Nahas SA, Butch AW, Du L, Gatti RA. Clinical chemistry. 2009 55:463-72. [PubMed:19147735] [PMC:PMC2980758]

Small-molecule drugs mimicking DNA damage: a new strategy for sensitizing tumors to radiotherapy. Quanz M, Berthault N, Roulin C, Roy M, Herbette A, Agrario C, Alberti C, Josserand V, Coll JL, Sastre-Garau X, Cosset JM, Larue L, Sun JS, Dutreix M. Clinical cancer research : an official journal of the American Association for Cancer Research. 2009 15:1308-16. [PubMed:19190126]

Inactivation of the Nijmegen breakage syndrome gene leads to excess centrosome duplication via the ATR/BRCA1 pathway. Shimada M, Sagae R, Kobayashi J, Habu T, Komatsu K. Cancer research. 2009 69:1768-75. [PubMed:19244116]

Temporal regulation of the Mre11-Rad50-Nbs1 complex during adenovirus infection. Karen KA, Hoey PJ, Young CS, Hearing P. Journal of virology. 2009 83:4565-73. [PubMed:19244322] [PMC:PMC2668508]

Adeno-associated virus replication induces a DNA damage response coordinated by DNA-dependent protein kinase. Schwartz RA, Carson CT, Schuberth C, Weitzman MD. Journal of virology. 2009 83:6269-78. [PubMed:19339345] [PMC:PMC2687378]

Ionizing radiation induces ataxia telangiectasia mutated-dependent checkpoint signaling and G(2) but not G(1) cell cycle arrest in pluripotent human embryonic stem cells. Momcilovi O, Choi S, Varum S, Bakkenist C, Schatten G, Navara C. Stem cells (Dayton, Ohio). 2009 27:1822-35. (WB) [PubMed:19544417] [PMC:PMC3340923]

Differential cellular responses to prolonged LDR-IR in MLH1-proficient and MLH1-deficient colorectal cancer HCT116 cells. Yan T, Seo Y, Kinsella TJ. Clinical cancer research : an official journal of the American Association for Cancer Research. 2009 15:6912-20. [PubMed:19861440] [PMC:PMC2783277]

Autophosphorylation at serine 1981 stabilizes ATM at DNA damage sites. So S, Davis AJ, Chen DJ. The Journal of cell biology. 2009 187:977-90. [PubMed:20026654] [PMC:PMC2806275]

Identification of increased NBS1 expression as a prognostic marker of squamous cell carcinoma of the oral cavity. Hsu DS, Chang SY, Liu CJ, Tzeng CH, Wu KJ, Kao JY, Yang MH. Cancer science. 2010 101:1029-37. (IHC, WB; Human) [PubMed:20175780]

Human T-lymphotropic type 1 virus p30 inhibits homologous recombination and favors unfaithful DNA repair. Baydoun HH, Pancewicz J, Nicot C. Blood. 2011 117:5897-906. [PubMed:21427292] [PMC:PMC3112037]

Ataxia telangiectasia mutated- and Rad3-related kinase drives both the early and the late DNA-damage response to the monofunctional antitumour alkylator S23906. Soares DG, Battistella A, Rocca CJ, Matuo R, Henriques JA, Larsen AK, Escargueil AE. The Biochemical journal. 2011 437:63-73. (ICC; Human) [PubMed:21470188]

Genomic instability, defective spermatogenesis, immunodeficiency, and cancer in a mouse model of the RIDDLE syndrome. Bohgaki T, Bohgaki M, Cardoso R, Panier S, Zeegers D, Li L, Stewart GS, Sanchez O, Hande MP, Durocher D, Hakem A, Hakem R. PLoS genetics. 2011 7:e1001381. (WB; Mouse) [PubMed:21552324] [PMC:PMC3084200]

Purkinje cell degeneration in pcd mice reveals large scale chromatin reorganization and gene silencing linked to defective DNA repair. Baltans FC, Casafont I, Lafarga V, Weruaga E, Alonso JR, Berciano MT, Lafarga M. The Journal of biological chemistry. 2011 286:28287-302. [PubMed:21700704] [PMC:PMC3151073]

ATM protein-dependent phosphorylation of Rad50 protein regulates DNA repair and cell cycle control. Gatei M, Jakob B, Chen P, Kijas AW, Becherel OJ, Gueven N, Birrell G, Lee JH, Paull TT, Lerenthal Y, Fazry S, Taucher-Scholz G, Kalb R, Schindler D, Waltes R, Drk T, Lavin MF. The Journal of biological chemistry. 2011 286:31542-56. (WB; Human) [PubMed:21757780] [PMC:PMC3173097]

Human Mre11/human Rad50/Nbs1 and DNA ligase IIIalpha/XRCC1 protein complexes act together in an alternative nonhomologous end joining pathway. Della-Maria J, Zhou Y, Tsai MS, Kuhnlein J, Carney JP, Paull TT, Tomkinson AE. The Journal of biological chemistry. 2011 286:33845-53. [PubMed:21816818] [PMC:PMC3190819]

Prognostic significance of NBS1 and Snail expression in esophageal squamous cell carcinoma. Kuo KT, Chou TY, Hsu HS, Chen WL, Wang LS. Annals of surgical oncology. 2012 S549-57. (IHC; Human) [PubMed:21881923]

Adeno-associated virus type 2 modulates the host DNA damage response induced by herpes simplex virus 1 during coinfection. Vogel R, Seyffert M, Strasser R, de Oliveira AP, Dresch C, Glauser DL, Jolinon N, Salvetti A, Weitzman MD, Ackermann M, Fraefel C. Journal of virology. 2012 86:143-55. [PubMed:22013059] [PMC:PMC3255894]

Oncolytic virus-mediated manipulation of DNA damage responses: synergy with chemotherapy in killing glioblastoma stem cells. Kanai R, Rabkin SD, Yip S, Sgubin D, Zaupa CM, Hirose Y, Louis DN, Wakimoto H, Martuza RL. Journal of the National Cancer Institute. 2012 104:42-55. [PubMed:22173583] [PMC:PMC3250384]

Effects of depletion of dihydropyrimidine dehydrogenase on focus formation and RPA phosphorylation. Someya M, Sakata K, Matsumoto Y, Tauchi H, Kai M, Hareyama M, Fukushima M. Journal of radiation research. 2012 53:250-6. (ICC; Human) [PubMed:22510597]

Adenovirus regulates sumoylation of Mre11-Rad50-Nbs1 components through a paralog-specific mechanism. Sohn SY, Hearing P. Journal of virology. 2012 86:9656-65. (ICC, WB; Human) [PubMed:22740413] [PMC:PMC3446602]

Chemical genetics reveals a specific requirement for Cdk2 activity in the DNA damage response and identifies Nbs1 as a Cdk2 substrate in human cells. Wohlbold L, Merrick KA, De S, Amat R, Kim JH, Larochelle S, Allen JJ, Zhang C, Shokat KM, Petrini JH, Fisher RP. PLoS genetics. 2012 8:e1002935. (WB, IP; Human) [PubMed:22927831] [PMC:PMC3426557]

A structural basis for the assembly and functions of a viral polymer that inactivates multiple tumor suppressors. Ou HD, Kwiatkowski W, Deerinck TJ, Noske A, Blain KY, Land HS, Soria C, Powers CJ, May AP, Shu X, Tsien RY, Fitzpatrick JA, Long JA, Ellisman MH, Choe S, O'Shea CC. Cell. 2012 151:304-19. (ICC; Human) [PubMed:23063122]

DUSP6/MKP3 is overexpressed in papillary and poorly differentiated thyroid carcinoma and contributes to neoplastic properties of thyroid cancer cells. Degl'Innocenti D, Romeo P, Tarantino E, Sensi M, Cassinelli G, Catalano V, Lanzi C, Perrone F, Pilotti S, Seregni E, Pierotti MA, Greco A, Borrello MG. Endocrine-related cancer. 2013 20:23-37. (WB; Human) [PubMed:23132790]

CtIP is required to initiate replication-dependent interstrand crosslink repair. Duquette ML, Zhu Q, Taylor ER, Tsay AJ, Shi LZ, Berns MW, McGowan CH. PLoS genetics. 2012 8:e1003050. (ICC; Human) [PubMed:23144634] [PMC:PMC3493458]

Mitochondrial dysfunction in a novel form of autosomal recessive ataxia. Murad NA, Cullen JK, McKenzie M, Ryan MT, Thorburn D, Gueven N, Kobayashi J, Birrell G, Yang J, Drk T, Becherel O, Grattan-Smith P, Lavin MF. Mitochondrion. 2013 13:235-45. (WB; Human) [PubMed:23178371]

Competition between NBS1 and ATMIN controls ATM signaling pathway choice. Zhang T, Penicud K, Bruhn C, Loizou JI, Kanu N, Wang ZQ, Behrens A. Cell reports. 2012 2:1498-504. (ICC, WB; Mouse) [PubMed:23219553]

Mutant fusion proteins with enhanced fusion activity promote measles virus spread in human neuronal cells and brains of suckling hamsters. Watanabe S, Shirogane Y, Suzuki SO, Ikegame S, Koga R, Yanagi Y. Journal of virology. 2013 87:2648-59. (ICC, IHC; Hamster) [PubMed:23255801] [PMC:PMC3571373]

VRK1 phosphorylates and protects NBS1 from ubiquitination and proteasomal degradation in response to DNA damage. Monsalve DM, Campillo-Marcos I, Salzano M, Sanz-García M, Cantarero L, Lazo PA. Biochimica et biophysica acta. 2016 1863:760-9. (ICC; Human) [Full Text Article] [PubMed:26869104]

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Gene Name:	nibrin
Synonyms:	NBN, ATV, AT-V2, Nibrin, NBS1, AT-V1, NBS, p95
Target Sequences:	NM_002485 NP_002476.2 O60934

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NBN / Nibrin

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Publications (95)

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