ABSTRACT Fanconi anaemia (FA) and Nijmegen breakage syndrome (NBS) are autosomal recessive chromosome instability syndromes with distinct clinical phenotypes. Cells from individuals affected

with FA are hypersensitive to mitomycin C (MMC), and cells from those with NBS are hypersensitive to ionizing radiation. Here we report that both NBS cell lines and individuals with NBS are

hypersensitive to MMC, indicating that there may be functional linkage between FA and NBS. In wild-type cells, MMC activates the colocalization of the FA subtype D2 protein (FANCD2) and

NBS1 protein in subnuclear foci. Ionizing radiation activates the ataxia telangiectasia kinase (ATM)-dependent and NBS1-dependent phosphorylation of FANCD2, resulting in an S-phase

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SIMILAR CONTENT BEING VIEWED BY OTHERS FANCONI ANEMIA PROTEINS PARTICIPATE IN A BREAK-INDUCED-REPLICATION-LIKE PATHWAY TO COUNTER REPLICATION STRESS Article 10 June 2021 FANCONI ANEMIA

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Download references ACKNOWLEDGEMENTS We thank A. M. R. Taylor for the ATLD lymphoblasts; H. Joenje for the EUFA1020 lymphoblasts; and C. Cale and P. Telfer for clinical information. This

work was supported by grants from the NIH (to A.D.D.). AUTHOR INFORMATION AUTHORS AND AFFILIATIONS * Department of Pediatric Oncology, Dana-Farber Cancer Institute, 44 Binney Street, Boston,

02115, MA, USA Koji Nakanishi, Toshiyasu Taniguchi, Lisa A. Moreau, Maria Stotsky & Alan D. D'Andrea * Center for Blood Research, Harvard Medical School, 200 Longwood Avenue,

Boston, 02115, MA, USA Velvizhi Ranganathan & David T. Weaver * Department of Haematology, Great Ormond Street Hospital, Great Ormond Street, London, WC1N 3JH, UK Helen V. New * Division

of Medical and Molecular Genetics, Guy's, King's, and St Thomas School of Medicine, 8th Floor, Guy's Tower, London, SE1 9RT, UK Christopher G. Mathew * Department of

Hematology-Oncology, St. Jude Children's Research Hospital, 323 North Lauderdale Street, Memphis, 38105, TN, USA Michael B. Kastan Authors * Koji Nakanishi View author publications You

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can also search for this author inPubMed Google Scholar CORRESPONDING AUTHOR Correspondence to Alan D. D'Andrea. ETHICS DECLARATIONS COMPETING INTERESTS The authors declare no competing

financial interests. RIGHTS AND PERMISSIONS Reprints and permissions ABOUT THIS ARTICLE CITE THIS ARTICLE Nakanishi, K., Taniguchi, T., Ranganathan, V. _et al._ Interaction of FANCD2 and

NBS1 in the DNA damage response. _Nat Cell Biol_ 4, 913–920 (2002). https://doi.org/10.1038/ncb879 Download citation * Received: 03 June 2002 * Revised: 14 August 2002 * Accepted: 17

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