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2. Cleavage of the BRCT tandem domains of nibrin by the 657del5 mutation affects the DNA damage response less than the Arg215Trp mutation. Mendez G; Cilli D; Berardinelli F; Viganotti M; Ascenzi P; Tanzarella C; Antoccia A; di Masi A IUBMB Life; 2012 Oct; 64(10):853-61. PubMed ID: 22941933 [TBL] [Abstract][Full Text] [Related]
4. Hsp90α regulates ATM and NBN functions in sensing and repair of DNA double-strand breaks. Pennisi R; Antoccia A; Leone S; Ascenzi P; di Masi A FEBS J; 2017 Aug; 284(15):2378-2395. PubMed ID: 28631426 [TBL] [Abstract][Full Text] [Related]
5. Human RAD50 deficiency in a Nijmegen breakage syndrome-like disorder. Waltes R; Kalb R; Gatei M; Kijas AW; Stumm M; Sobeck A; Wieland B; Varon R; Lerenthal Y; Lavin MF; Schindler D; Dörk T Am J Hum Genet; 2009 May; 84(5):605-16. PubMed ID: 19409520 [TBL] [Abstract][Full Text] [Related]
6. Identification of the interactors of human nibrin (NBN) and of its 26 kDa and 70 kDa fragments arising from the NBN 657del5 founder mutation. Cilli D; Mirasole C; Pennisi R; Pallotta V; D'Alessandro A; Antoccia A; Zolla L; Ascenzi P; di Masi A PLoS One; 2014; 9(12):e114651. PubMed ID: 25485873 [TBL] [Abstract][Full Text] [Related]
8. Bone Marrow Failure and Immunodeficiency Associated with Human RAD50 Variants. Takagi M; Hoshino A; Bousset K; Röddecke J; Martin HL; Folcut I; Tomomasa D; Yang X; Kobayashi J; Sakata N; Yoshida K; Miyano S; Ogawa S; Kojima S; Morio T; Dörk T; Kanegane H J Clin Immunol; 2023 Nov; 43(8):2136-2145. PubMed ID: 37794136 [TBL] [Abstract][Full Text] [Related]
9. Clinical variability and expression of the NBN c.657del5 allele in Nijmegen Breakage Syndrome. Lins S; Kim R; Krüger L; Chrzanowska KH; Seemanova E; Digweed M Gene; 2009 Nov; 447(1):12-7. PubMed ID: 19635536 [TBL] [Abstract][Full Text] [Related]
10. Nbn and atm cooperate in a tissue and developmental stage-specific manner to prevent double strand breaks and apoptosis in developing brain and eye. Rodrigues PM; Grigaravicius P; Remus M; Cavalheiro GR; Gomes AL; Rocha-Martins M; Frappart L; Reuss D; McKinnon PJ; von Deimling A; Martins RA; Frappart PO PLoS One; 2013; 8(7):e69209. PubMed ID: 23935957 [TBL] [Abstract][Full Text] [Related]
11. Human RAD50 deficiency: Confirmation of a distinctive phenotype. Ragamin A; Yigit G; Bousset K; Beleggia F; Verheijen FW; de Wit MY; Strom TM; Dörk T; Wollnik B; Mancini GMS Am J Med Genet A; 2020 Jun; 182(6):1378-1386. PubMed ID: 32212377 [TBL] [Abstract][Full Text] [Related]
13. An essential function for NBS1 in the prevention of ataxia and cerebellar defects. Frappart PO; Tong WM; Demuth I; Radovanovic I; Herceg Z; Aguzzi A; Digweed M; Wang ZQ Nat Med; 2005 May; 11(5):538-44. PubMed ID: 15821748 [TBL] [Abstract][Full Text] [Related]
14. Three new cases of ataxia-telangiectasia-like disorder: No impairment of the ATM pathway, but S-phase checkpoint defect. Fiévet A; Bellanger D; Valence S; Mobuchon L; Afenjar A; Giuliano F; Dubois d'Enghien C; Parfait B; Pedespan JM; Auger N; Rieunier G; Collet A; Burglen L; Stoppa-Lyonnet D; Stern MH Hum Mutat; 2019 Oct; 40(10):1690-1699. PubMed ID: 31033087 [TBL] [Abstract][Full Text] [Related]
15. 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 Mol Cancer Res; 2003 Jul; 1(9):674-81. PubMed ID: 12861053 [TBL] [Abstract][Full Text] [Related]
16. The R215W mutation in NBS1 impairs gamma-H2AX binding and affects DNA repair: molecular bases for the severe phenotype of 657del5/R215W Nijmegen breakage syndrome patients. di Masi A; Viganotti M; Polticelli F; Ascenzi P; Tanzarella C; Antoccia A Biochem Biophys Res Commun; 2008 May; 369(3):835-40. PubMed ID: 18328813 [TBL] [Abstract][Full Text] [Related]
17. Nbn gene inactivation in the CNS of mouse inhibits the myelinating ability of the mature cortical oligodendrocytes. Liu B; Chen X; Wang ZQ; Tong WM Glia; 2014 Jan; 62(1):133-44. PubMed ID: 24272708 [TBL] [Abstract][Full Text] [Related]
18. Analysis of ataxia-telangiectasia mutated (ATM)- and Nijmegen breakage syndrome (NBS)-regulated gene expression patterns. Jang ER; Lee JH; Lim DS; Lee JS J Cancer Res Clin Oncol; 2004 Apr; 130(4):225-34. PubMed ID: 14745549 [TBL] [Abstract][Full Text] [Related]
19. Loss of juxtaposition of RAG-induced immunoglobulin DNA ends is implicated in the precursor B-cell differentiation defect in NBS patients. van der Burg M; Pac M; Berkowska MA; Goryluk-Kozakiewicz B; Wakulinska A; Dembowska-Baginska B; Gregorek H; Barendregt BH; Krajewska-Walasek M; Bernatowska E; van Dongen JJ; Chrzanowska KH; Langerak AW Blood; 2010 Jun; 115(23):4770-7. PubMed ID: 20378756 [TBL] [Abstract][Full Text] [Related]
20. DNA damage in Nijmegen Breakage Syndrome cells leads to PARP hyperactivation and increased oxidative stress. Krenzlin H; Demuth I; Salewsky B; Wessendorf P; Weidele K; Bürkle A; Digweed M PLoS Genet; 2012; 8(3):e1002557. PubMed ID: 22396666 [TBL] [Abstract][Full Text] [Related] [Next] [New Search]