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Showing NBNNBS1 is a alias.

NBN

Nibrin · UniProt O60934

Length
754 aa
Mass
85.0 kDa
Annotated
2026-06-10
100 papers in source corpus 14 papers cited in narrative 14 extracted findings
Cross-family judge vs UniProt: Affinage preferred faithfulness: 7/8 claims corpus-supported (88%)

Mechanistic narrative

Synthesis pass · prose summary of the discoveries below

NBN (nibrin) is the adapter subunit of the MRE11/RAD50/NBN (MRN) complex, the sensor that detects DNA double-strand breaks (DSBs) and couples their repair to ATM-dependent checkpoint signaling (PMID:24396275). NBN is required for nuclear import of the MRE11-RAD50 core: loss of nibrin function leaves Rad50 cytoplasmic, and NBN-mutant alleles that reduce protein levels or disrupt the steady-state association redistribute both MRN and ATM from nucleus to cytoplasm (PMID:16648644, PMID:31729086). The physical NBN–MRE11 interaction is itself essential, as an allele with impaired Mre11 binding causes hematopoietic failure and T cell leukemia driven by compensatory MRE11 amplification (PMID:31285322). At breaks, ATM phosphorylates NBN to set the kinetics of MRN and ATM accumulation at DSB sites and enable timely repair: phospho-blocking mutations delay recruitment while phosphomimetic substitutions prolong it (PMID:23146902). NBN-mediated DSB processing is also required to resolve O6-methylguanine-derived lesions, since MGMT overexpression abolishes the temozolomide hypersensitivity of NBN-deficient cells (PMID:20729302). Beyond canonical repair, nibrin restrains reactive-oxygen-species production after DSB induction (PMID:19412544) and, in the developing CNS, governs oligodendrocyte precursor survival and proliferation through ATM-Chk2 and AKT/mTOR signaling, with its loss producing hypomyelination (PMID:24272991). NBN stability depends on the chaperone Hsp90α: pharmacological Hsp90α inhibition triggers polyubiquitination and proteasomal degradation of NBN and ATM, impairs MRE11/RAD50 nuclear localization, and attenuates downstream BRCA1 and CHK2 phosphorylation (PMID:28631426). Patient-derived truncating and missense variants that abolish NBN focus formation or reduce protein levels confer radiosensitivity, impaired downstream focus formation, and PARP-inhibitor hypersensitivity (PMID:36346689, PMID:24928521).

Mechanistic history

Synthesis pass · year-by-year structured walk · 10 steps
  1. 2002 Medium

    Established that the MRN complex physically organizes into DSB-associated nuclear structures together with PML and p53, linking MRN to checkpoint factors at damage sites.

    Evidence Immunofluorescence co-localization with γ-H2AX and co-IP of p53 with hMre11/PML after IR in human fibroblasts

    PMID:11896594

    Open questions at the time
    • Does not define NBN's specific contribution to PML/p53 recruitment
    • Co-localization does not establish direct NBN binding to p53 or PML
  2. 2006 High

    Resolved that nibrin is required for nuclear import of the MRE11-RAD50 core, defining NBN's role as the adapter delivering the complex into the nucleus.

    Evidence Genetic epistasis and Rad50 localization in Drosophila nbs mutants with ATM/ATR double-mutant analysis

    PMID:16648644

    Open questions at the time
    • Mechanism of NBN-mediated nuclear import (NLS usage, import machinery) not defined
    • Drosophila ortholog may not fully recapitulate human domain functions
  3. 2009 High

    Distinguished NBN from MRE11 in tissue-specific ATM signaling and revealed an unanticipated role for nibrin in restraining oxidative stress alongside DSB repair.

    Evidence Mouse conditional/null genetics with ATM-substrate immunoblotting and TUNEL; 2D-gel proteomics and ROS measurement in Nbn-null fibroblasts

    PMID:19171781 PMID:19412544

    Open questions at the time
    • Molecular basis of NBN's ROS regulation not identified
    • Whether oxidative phenotype is a direct NBN function or downstream of unrepaired DSBs unresolved
  4. 2010 High

    Showed that MRN-dependent DSB processing is the basis of cellular resistance to O6-methylguanine lesions, defining a specific lesion type whose repair requires NBN.

    Evidence Clonogenic and cell-death assays in NBN-mutant patient cells and melanoma siRNA knockdown with MGMT rescue epistasis

    PMID:20729302

    Open questions at the time
    • Step at which NBN acts on O6-methylguanine-derived DSBs not defined
    • Does not separate MRN nuclease activity from NBN adapter function
  5. 2012 High

    Demonstrated that ATM phosphorylation of NBN feeds back to control the kinetics of MRN and ATM accumulation at breaks, establishing NBN phosphorylation as a timing regulator of the DDR.

    Evidence Site-specific I-PpoI endonuclease in isogenic NBN phospho-mutant cells with ChIP at defined DSBs and repair kinetics assays

    PMID:23146902

    Open questions at the time
    • Identity of phospho-NBN binding partners mediating retention not defined
    • Structural basis of phosphorylation-dependent recruitment unknown
  6. 2013 Medium

    Extended NBN function to CNS development, showing nibrin loss triggers ATM-Chk2 apoptosis and AKT/mTOR proliferation defects in oligodendrocyte precursors causing hypomyelination.

    Evidence Nestin-Cre conditional Nbn knockout mouse with pathway immunoblotting and BDNF/NGF rescue

    PMID:24272991

    Open questions at the time
    • Direct molecular link between NBN and AKT/mTOR regulation not established
    • Whether OPC phenotype reflects DSB repair failure or a separate NBN function unclear
  7. 2014 Medium

    Mapped the NBN interactome and showed that a disease-derived FHA/BRCT1-containing p26 fragment aberrantly binds and inhibits PARP1, linking NBN truncation to persistent ROS and DSBs.

    Evidence Affinity-MS interactome from HEK293 with co-IP of p26-PARP1 and NAD+ activity assay; plus functional analysis of the p.R215W allele showing loss of NBN foci and PARP-inhibitor hypersensitivity

    PMID:24928521 PMID:25485873

    Open questions at the time
    • Physiological relevance of p26-PARP1 inhibition versus loss of full-length NBN unresolved
    • Interactome partners not validated by reciprocal IP
  8. 2017 High

    Identified NBN (and ATM) as Hsp90α clients, establishing chaperone-dependent stability as a prerequisite for NBN-mediated MRN nuclear localization and DDR signaling.

    Evidence Reciprocal Co-IP of Hsp90α with NBN/ATM, 17-AAG inhibition with proteasome rescue, fractionation, and phospho-readouts (BRCA1, CHK2)

    PMID:28631426

    Open questions at the time
    • Hsp90α binding region on NBN not mapped
    • Whether chaperone dependence is constitutive or damage-regulated not fully defined
  9. 2019 High

    Established that the NBN–MRE11 physical interaction is essential for tumor suppression and hematopoiesis, and that NBN sustains steady-state nuclear co-localization of MRN and ATM.

    Evidence Mouse Nbnmid8 binding-impaired allele with hematopoietic/tumor phenotyping and genomic analysis; human infertility variants producing p84-NBN with MRN/ATM cytoplasmic relocalization

    PMID:31285322 PMID:31729086

    Open questions at the time
    • How weakened NBN-MRE11 binding mechanistically drives leukemogenesis beyond MRE11 amplification unresolved
    • Determinants distinguishing infertility-only phenotypes from classic NBS not defined
  10. 2023 Medium

    Showed that truncating NBN variants yield alternative-translation fragments that retain MRE11 binding yet fail to support ATM-dependent signaling, linking partial NBN function to radiosensitivity.

    Evidence Immunoblot detection of a 45 kDa fragment, co-IP with MRE11, γ-irradiation survival, and phospho-KAP1 readout

    PMID:36346689

    Open questions at the time
    • Why MRE11-binding-competent fragments still fail to signal not mechanistically resolved
    • Single-lab, limited replication

Open questions

Synthesis pass · forward-looking unresolved questions
  • How NBN integrates its DSB-adapter role with oxidative-stress regulation and tissue-specific survival/proliferation signaling remains unresolved.
  • No molecular mechanism connecting NBN to ROS homeostasis
  • No structural model of phosphorylation-dependent MRN/ATM retention at breaks
  • Direct biochemical link between NBN and AKT/mTOR signaling unestablished

Mechanism profile

Synthesis pass · controlled-vocabulary classification · explore literature graph →
Molecular activity
GO:0060090 molecular adaptor activity 3 GO:0003677 DNA binding 2 GO:0140104 molecular carrier activity 2
Localization
GO:0000228 nuclear chromosome 3 GO:0005634 nucleus 3 GO:0005829 cytosol 1
Pathway
R-HSA-73894 DNA Repair 3 R-HSA-8953897 Cellular responses to stimuli 3 R-HSA-1640170 Cell Cycle 2
Complex memberships
MRE11/RAD50/NBN (MRN) complex

Evidence

Reading pass · 14 per-paper findings extracted from the source corpus
Year Finding Method Journal Conf PMIDs
2006 Drosophila Nbs mediates nuclear transport of the Mre11/Rad50 complex: Rad50 accumulates in nuclei of wild-type cells but not in nbs mutant cells, indicating Nbs is required for nuclear localization of the MRN complex. Epistasis analysis showed nbs functions in both ATM- and ATR-controlled telomere protection pathways, while all three MRN components function in a single pathway for DNA-damage-induced chromosome break repair. Genetic epistasis analysis in Drosophila nbs mutants; immunofluorescence/fractionation showing Rad50 nuclear localization; double-mutant analysis with tefu (ATM) and mei-41 (ATR) Genetics High 16648644
2009 In the nervous system, NBS1 (Nbs1) is required for normal ATM-dependent apoptosis after DNA damage: Nbs1ΔB/ΔB neural tissue shows defective ATM phosphorylation yet normal apoptosis, whereas Mre11ATLD1/ATLD1 tissue shows defective ATM activation and pronounced resistance to apoptosis. Genetic rescue experiments showed Mre11 and ATM backgrounds (but not Nbs1ΔB) rescue Lig4-induced microcephaly, placing Nbs1 and Mre11 in distinct sub-pathways for neural ATM signaling. Mouse conditional genetics; epistasis (Lig4 loss on Nbs1ΔB, Mre11ATLD1, Atm−/− backgrounds); immunoblotting for ATM substrates (Chk2, p53 phosphorylation); TUNEL apoptosis assay Genes & development High 19171781
2012 ATM phosphorylation of NBN regulates the kinetics of NBN and ATM accumulation at DNA double-strand break sites: phospho-blocking (Ser→Ala) mutations delayed recruitment of both NBN and ATM to DSBs, while phosphomimetic (Ser→Glu) substitutions increased and prolonged their accumulation. Full-length NBN was required for timely DSB repair. Direct protein transduction of site-specific endonuclease I-PpoI into isogenic cell lines expressing NBN phosphorylation mutants; chromatin immunoprecipitation at defined DSB sites; DSB repair kinetics assay Oncogene High 23146902
2002 After ionizing radiation, PML nuclear bodies associate with the hMre11/Rad50/NBS1 complex at sites of DNA double-strand breaks (marked by γ-H2AX), and ionizing radiation induces stable physical association of p53 with hMre11 and PML within these foci. Immunofluorescence co-localization with anti-γ-H2AX; co-immunoprecipitation of p53 with hMre11 and PML after IR in normal human fibroblasts Oncogene Medium 11896594
2017 NBN (nibrin) and ATM are clients of the molecular chaperone Hsp90α: inhibition of Hsp90α ATPase activity with 17-AAG causes polyubiquitination and proteasomal degradation of ATM and NBN (but not 53BP1, RAD50, or MRE11). Hsp90α–ATM and Hsp90α–NBN complexes exist in unstressed and irradiated cells. Upon IR-induced DSBs, ATM phosphorylates NBN, which then dissociates from Hsp90α and translocates to DSB sites. Hsp90α inhibition impairs nuclear localization of MRE11 and RAD50, attenuates DDR signaling (BRCA1, CHK2 phosphorylation), and slows DSB repair. Co-immunoprecipitation of Hsp90α with ATM and NBN; 17-AAG inhibitor treatment with proteasome inhibitor rescue; immunofluorescence for MRE11/RAD50 nuclear localization; immunoblotting for phospho-NBN, phospho-BRCA1, phospho-CHK2; subcellular fractionation The FEBS journal High 28631426
2014 Full-length NBN protein interacts with a broad set of partners involved in DNA damage response, ROS scavenging, and protein folding. The 26 kDa fragment (p26, containing FHA/BRCT1 domains) arising from the NBS founder mutation 657del5 interacts with PARP1 after irradiation, and this interaction inhibits PARP1 activity (measured by NAD+ levels) and is associated with persistence of ROS and DSBs at 24 h post-IR. Affinity chromatography from transiently transfected HEK293 cells expressing full-length NBN, p26, or p70; SDS-PAGE separation and shotgun MS/MS protein identification; co-immunoprecipitation of p26 with PARP1; NAD+ assay for PARP1 activity PloS one Medium 25485873
2010 NBN (nibrin) is required for cellular resistance to O6-methylguanine-inducing agents (e.g., temozolomide, MNNG): NBN-mutant NBS cells are hypersensitive to these agents through increased apoptosis and necrosis. MGMT over-expression abrogated the hypersensitivity, indicating the response is specifically triggered by O6-methylguanine lesions requiring DSB processing by the MRN complex. siRNA knockdown of NBN in melanoma cells recapitulated the temozolomide sensitization. Clonogenic survival and cell death assays in NBN-mutant patient fibroblasts and lymphoblastoid cells; MGMT expression rescue experiment; siRNA knockdown of NBN in melanoma cells followed by temozolomide treatment; apoptosis/necrosis quantification Molecular pharmacology High 20729302
2019 Physical interaction between Nbn and the Mre11-Rad50 core is required for tumor suppression and normal hematopoiesis: a mouse Nbn allele (Nbnmid8) with severely impaired Mre11 binding caused profound hematopoietic defects, B cell development blockage, and rapid T cell leukemia. Leukemias showed focal amplification of 9qA2 causing MRE11 overexpression, interpreted as compensation for the weakened Mre11-Nbn interaction. Mouse genetics (conditional hematopoietic expression of Nbnmid8); flow cytometry of thymus and bone marrow cellularity; genomic analysis of tumors; copy number and gene expression analysis Proceedings of the National Academy of Sciences of the United States of America High 31285322
2019 NBN variants causing isolated infertility produce an alternative p84-NBN protein (from in-frame exon 4-5 skipping) that retains the FHA domain but shows dramatically reduced protein levels and causes relocalization of the MRN complex from the nucleus to the cytoplasm. ATM also shifts from nucleus to cytoplasm in these cells, suggesting steady-state ATM–MRN nuclear co-localization depends on NBN. ATM pathway activation was partially preserved, and cell cycle checkpoint defects were milder than in typical NBS. Transcript analysis (RT-PCR, sequencing); immunoblotting for NBN protein variants; immunofluorescence for subcellular localization of MRN complex and ATM; ATM pathway activation assay (camptothecin treatment); cell cycle checkpoint analysis Human mutation Medium 31729086
2013 The MRN complex (MRE11/RAD50/NBN) functions as a DNA double-strand break sensor and initiates DNA repair by activating ATM kinase, which coordinates repair with p53-dependent cell cycle checkpoint arrest. NBN contributes the FHA and BRCT domains for interaction with phosphorylated substrates and acts as the adapter subunit linking the MRE11 nuclease/RAD50 ATPase core to downstream checkpoint signaling. Review synthesizing functional genetic and biochemical studies (co-immunoprecipitation, domain deletion analyses, patient cell studies cited across the field) Current genomics Low 24396275
2023 Two novel NBN truncating variants (p.L19* and p.N71fs) produce a 45 kDa alternative-translation fragment that maintains binding to MRE11. Cells expressing these fragments show higher sensitivity to γ-irradiation and lower levels of radiation-induced KAP1 phosphorylation, indicating impaired ATM-dependent DDR signaling. Immunoblotting for alternative translation product; co-immunoprecipitation of 45 kDa fragment with MRE11; γ-irradiation survival assay; immunoblotting for phospho-KAP1 Clinical cancer research Medium 36346689
2014 The NBN p.R215W missense mutation in the hemizygous state impairs NBN protein levels and abolishes formation of NBN foci at DNA damage sites after irradiation. Cells are highly radiosensitive, show impaired γH2AX, MDC1, and 53BP1 focus formation, and are hypersensitive to PARP1 inhibition, consistent with a combined NBN/BRCA1 deficiency. ATM signaling was largely unaffected despite NBN focus deficiency. Immunocytochemistry for NBN, γH2AX, MDC1, 53BP1 foci after IR; immunoblotting for NBN protein; clonogenic radiosensitivity assay; flow cytometry for cell cycle; impedance measurement for PARP1 inhibitor sensitivity BMC cancer Medium 24928521
2009 Nibrin-deficient mouse cells show increased production of reactive oxygen species following induction of DSBs, implicating NBN in oxidative stress regulation in addition to DSB repair. Proteomic analysis revealed significant alterations in proteins involved in oxidative stress and cellular redox homeostasis in nibrin null mutant mice. Conditional null mutant mouse model; 2D-gel proteomics (~8,000 proteins resolved); ROS measurement in Nbn null mutant fibroblasts after DSB induction PloS one Medium 19412544
2013 Nbn gene inactivation in the CNS leads to defective proliferation and enhanced apoptosis of oligodendrocyte precursor cells (OPCs) causing hypomyelination of corpus callosum. ATM-Chk2 signaling drives apoptosis while AKT/mTOR signaling controls proliferation of Nbn-deficient OPCs; BDNF/NGF stimulation attenuates oxidative stress and restores OPC proliferation via AKT/mTOR/P70S6K. Nestin-Cre conditional Nbn knockout mouse; immunohistochemistry/immunofluorescence for myelin and OPC markers; immunoblotting for ATM-Chk2 and AKT/mTOR pathway components; BDNF/NGF rescue experiments Journal of neuroscience research Medium 24272991

Source papers

Stage 0 corpus · 100 papers · ranked by NIH iCite citations
Year Title Journal Citations PMID
2006 Plant NBS-LRR proteins in pathogen sensing and host defense. Nature immunology 462 17110940
2015 Germline Mutations in the BRIP1, BARD1, PALB2, and NBN Genes in Women With Ovarian Cancer. Journal of the National Cancer Institute 330 26315354
2004 Plant disease resistance protein signaling: NBS-LRR proteins and their partners. Current opinion in plant biology 322 15231261
2002 Interaction between domains of a plant NBS-LRR protein in disease resistance-related cell death. The EMBO journal 312 12198153
2008 Recent duplications dominate NBS-encoding gene expansion in two woody species. Molecular genetics and genomics : MGG 294 18563445
2013 Plant nucleotide binding site-leucine-rich repeat (NBS-LRR) genes: active guardians in host defense responses. International journal of molecular sciences 195 23549266
2012 Nijmegen breakage syndrome (NBS). Orphanet journal of rare diseases 174 22373003
2002 PML NBs associate with the hMre11 complex and p53 at sites of irradiation induced DNA damage. Oncogene 155 11896594
2002 Diversity, distribution, and ancient taxonomic relationships within the TIR and non-TIR NBS-LRR resistance gene subfamilies. Journal of molecular evolution 134 11956693
2013 miR482 regulation of NBS-LRR defense genes during fungal pathogen infection in cotton. PloS one 133 24391949
2008 Regulation of apoptosis by PML and the PML-NBs. Oncogene 127 18931695
2003 Nbn heterozygosity renders mice susceptible to tumor formation and ionizing radiation-induced tumorigenesis. Cancer research 119 14612522
2004 Identification of I50L as the signature atazanavir (ATV)-resistance mutation in treatment-naive HIV-1-infected patients receiving ATV-containing regimens. The Journal of infectious diseases 118 15122516
2015 Identification and distribution of the NBS-LRR gene family in the Cassava genome. BMC genomics 106 25948536
2014 The stripe rust resistance gene Yr10 encodes an evolutionary-conserved and unique CC-NBS-LRR sequence in wheat. Molecular plant 105 25336565
2017 The NBS-LRR architectures of plant R-proteins and metazoan NLRs evolved in independent events. Proceedings of the National Academy of Sciences of the United States of America 95 28096345
2015 Pi64, Encoding a Novel CC-NBS-LRR Protein, Confers Resistance to Leaf and Neck Blast in Rice. Molecular plant-microbe interactions : MPMI 95 25650828
2006 Structural and genomic properties of the hyperthermophilic archaeal virus ATV with an extracellular stage of the reproductive cycle. Journal of molecular biology 91 16677670
2009 Differential DNA damage signaling accounts for distinct neural apoptotic responses in ATLD and NBS. Genes & development 81 19171781
2011 Transcriptional analysis in high-anthocyanin tomatoes reveals synergistic effect of Aft and atv genes. Journal of plant physiology 73 20888667
2007 Cancer risk of heterozygotes with the NBN founder mutation. Journal of the National Cancer Institute 69 18073374
2015 Extreme expansion of NBS-encoding genes in Rosaceae. BMC genetics 54 25935646
2019 The Gossypium hirsutum TIR-NBS-LRR gene GhDSC1 mediates resistance against Verticillium wilt. Molecular plant pathology 49 30957942
2008 PML NBs (ND10) and Daxx: from nuclear structure to protein function. Frontiers in bioscience : a journal and virtual library 48 18508722
2003 Barley disease resistance gene analogs of the NBS-LRR class: identification and mapping. Molecular genetics and genomics : MGG 48 12715163
2019 Genome-wide characterization revealed role of NBS-LRR genes during powdery mildew infection in Vitis vinifera. Genomics 46 30802599
2006 The Drosophila Nbs protein functions in multiple pathways for the maintenance of genome stability. Genetics 46 16648644
2002 Characterization of expressed NBS-LRR resistance gene candidates from common bean. TAG. Theoretical and applied genetics. Theoretische und angewandte Genetik 41 12582850
2016 The Eucalyptus grandis NBS-LRR Gene Family: Physical Clustering and Expression Hotspots. Frontiers in plant science 40 26793216
2014 Dynamic evolution of NBS-LRR genes in bread wheat and its progenitors. Molecular genetics and genomics : MGG 40 25475390
2001 Origin, diversity and evolution of NBS-type disease-resistance gene homologues in coffee trees (Coffea L.). Molecular genetics and genomics : MGG 40 11459185
2018 Over-expression of GmKR3, a TIR-NBS-LRR type R gene, confers resistance to multiple viruses in soybean. Plant molecular biology 39 30535849
2020 Mutations in ATM, NBN and BRCA2 predispose to aggressive prostate cancer in Poland. International journal of cancer 36 32875559
2020 Genome-Wide Identification and Evolutionary Analysis of NBS-LRR Genes From Dioscorea rotundata. Frontiers in genetics 33 32457809
2019 A CC-NBS-LRR gene induces hybrid lethality in cotton. Journal of experimental botany 32 31270546
2003 Chromosome instability and nibrin protein variants in NBS heterozygotes. European journal of human genetics : EJHG 32 12708449
2018 Rpp1 Encodes a ULP1-NBS-LRR Protein That Controls Immunity to Phakopsora pachyrhizi in Soybean. Molecular plant-microbe interactions : MPMI 29 30303765
2017 Hsp90α regulates ATM and NBN functions in sensing and repair of DNA double-strand breaks. The FEBS journal 29 28631426
2014 NBN gain is predictive for adverse outcome following image-guided radiotherapy for localized prostate cancer. Oncotarget 29 25415046
2008 Transcriptional response of Mexican axolotls to Ambystoma tigrinum virus (ATV) infection. BMC genomics 27 18937860
2023 Interplay between PML NBs and HIRA for H3.3 dynamics following type I interferon stimulus. eLife 25 37227756
2023 Two functional CC-NBS-LRR proteins from rye chromosome 6RS confer differential age-related powdery mildew resistance to wheat. Plant biotechnology journal 24 38153293
2012 The potential link between PML NBs and ICP0 in regulating lytic and latent infection of HSV-1. Protein & cell 23 22544561
2022 Genome-Wide Identification and Characterization of the CC-NBS-LRR Gene Family in Cucumber (Cucumis sativus L.). International journal of molecular sciences 22 35563438
2021 Cotton CC-NBS-LRR Gene GbCNL130 Confers Resistance to Verticillium Wilt Across Different Species. Frontiers in plant science 22 34567025
2021 Natural history of children and adults with phenylketonuria in the NBS-PKU Connect registry. Molecular genetics and metabolism 22 34654619
2020 NOSH-aspirin (NBS-1120) inhibits pancreatic cancer cell growth in a xenograft mouse model: Modulation of FoxM1, p53, NF-κB, iNOS, caspase-3 and ROS. Biochemical pharmacology 22 32061771
2019 Identification and expression profiling analysis of NBS-LRR genes involved in Fusarium oxysporum f.sp. conglutinans resistance in cabbage. 3 Biotech 22 31065502
2022 PM2b, a CC-NBS-LRR protein, interacts with TaWRKY76-D to regulate powdery mildew resistance in common wheat. Frontiers in plant science 21 36388562
2020 NLGenomeSweeper: A Tool for Genome-Wide NBS-LRR Resistance Gene Identification. Genes 21 32245073
2018 Inherited NBN Mutations and Prostate Cancer Risk and Survival. Cancer research and treatment 21 30590007
2012 NBN phosphorylation regulates the accumulation of MRN and ATM at sites of DNA double-strand breaks. Oncogene 21 23146902
2021 Identification and Characterization of NBS Resistance Genes in Akebia trifoliata. Frontiers in plant science 20 34777439
2023 NBN Pathogenic Germline Variants are Associated with Pan-Cancer Susceptibility and In Vitro DNA Damage Response Defects. Clinical cancer research : an official journal of the American Association for Cancer Research 19 36346689
2021 Genome-wide identification and characterization of NBS-encoding genes in Raphanus sativus L. and their roles related to Fusarium oxysporum resistance. BMC plant biology 19 33461498
2016 Association of the nibrin gene (NBN) variants with breast cancer. Biomedical reports 19 26998278
2013 NBN Gene Polymorphisms and Cancer Susceptibility: A Systemic Review. Current genomics 19 24396275
2024 Functional characterization of NBS-LRR genes reveals an NBS-LRR gene that mediates resistance against Fusarium wilt. BMC biology 18 38408951
2023 Importance of Germline and Somatic Alterations in Human MRE11, RAD50, and NBN Genes Coding for MRN Complex. International journal of molecular sciences 18 36982687
2020 A Rice NBS-ARC Gene Conferring Quantitative Resistance to Bacterial Blight Is Regulated by a Pathogen Effector-Inducible miRNA. Molecular plant 18 32966899
2014 Identification of the interactors of human nibrin (NBN) and of its 26 kDa and 70 kDa fragments arising from the NBN 657del5 founder mutation. PloS one 18 25485873
2021 Genome-Wide Identification and Analysis of CC-NBS-LRR Family in Response to Downy Mildew and Black Rot in Chinese Cabbage. International journal of molecular sciences 17 33924035
2021 Ne2, a typical CC-NBS-LRR-type gene, is responsible for hybrid necrosis in wheat. The New phytologist 17 34160845
2023 IR-820@NBs Combined with MG-132 Enhances the Anti-Hepatocellular Carcinoma Effect of Sonodynamic Therapy. International journal of nanomedicine 15 37933299
2022 RppM, Encoding a Typical CC-NBS-LRR Protein, Confers Resistance to Southern Corn Rust in Maize. Frontiers in plant science 15 35903220
2021 Detecting Variants in the NBN Gene While Testing for Hereditary Breast Cancer: What to Do Next? International journal of molecular sciences 15 34072463
2017 Salicylic acid and broad spectrum of NBS-LRR family genes are involved in SMV-soybean interactions. Plant physiology and biochemistry : PPB 15 29232653
2016 The Slavic NBN Founder Mutation: A Role for Reproductive Fitness? PloS one 15 27936167
2014 Functional deficiency of NBN, the Nijmegen breakage syndrome protein, in a p.R215W mutant breast cancer cell line. BMC cancer 15 24928521
2013 Germline variants in MRE11/RAD50/NBN complex genes in childhood leukemia. BMC cancer 15 24093751
2011 AAA ATPase p529 of Acidianus two-tailed virus ATV and host receptor recognition. Virology 15 21982819
2010 Nijmegen breakage syndrome protein (NBN) causes resistance to methylating anticancer drugs such as temozolomide. Molecular pharmacology 15 20729302
2023 On the interplay between lipids and asymmetric dynamics of an NBS degenerate ABC transporter. Communications biology 14 36737455
2022 Filamentous nuclear actin regulation of PML NBs during the DNA damage response is deregulated by prelamin A. Cell death & disease 14 36522328
2020 Mutation Spectra of the MRN (MRE11, RAD50, NBS1/NBN) Break Sensor in Cancer Cells. Cancers 14 33339169
2018 Genome-scale examination of NBS-encoding genes in blueberry. Scientific reports 14 29467425
2018 OsCML16 interacts with a novel CC-NBS-LRR protein OsPi304 in the Ca2+/Mg2+ dependent and independent manner in rice. Biochemical and biophysical research communications 14 30190132
2023 An NBS-LRR protein in the Rpp1 locus negates the dominance of Rpp1-mediated resistance against Phakopsora pachyrhizi in soybean. The Plant journal : for cell and molecular biology 13 36424366
2023 Is Our Newborn Screening Working Well? A Literature Review of Quality Requirements for Newborn Blood Spot Screening (NBS) Infrastructure and Procedures. International journal of neonatal screening 13 37489488
2021 Identification and Cloning of a CC-NBS-NBS-LRR Gene as a Candidate of Pm40 by Integrated Analysis of Both the Available Transcriptional Data and Published Linkage Mapping. International journal of molecular sciences 13 34638580
2020 Structure and function analysis of a CC-NBS-LRR protein AT1G12290. Biochemical and biophysical research communications 13 33272575
2013 DNA damage and oxidative injury are associated with hypomyelination in the corpus callosum of newborn Nbn(CNS-del) mice. Journal of neuroscience research 13 24272991
2009 Clinical variability and expression of the NBN c.657del5 allele in Nijmegen Breakage Syndrome. Gene 13 19635536
2022 SUMOylation regulates the number and size of promyelocytic leukemia-nuclear bodies (PML-NBs) and arsenic perturbs SUMO dynamics on PML by insolubilizing PML in THP-1 cells. Archives of toxicology 12 35001170
2019 Therapeutic Intranasal Vaccine HB-ATV-8 Prevents Atherogenesis and Non-alcoholic Fatty Liver Disease in a Pig Model of Atherosclerosis. Archives of medical research 12 30792163
2019 Nbn-Mre11 interaction is required for tumor suppression and genomic integrity. Proceedings of the National Academy of Sciences of the United States of America 12 31285322
2019 Allelic modification of breast cancer risk in women with an NBN mutation. Breast cancer research and treatment 12 31410679
2018 Segmental and Tandem Duplications Driving the Recent NBS-LRR Gene Expansion in the Asparagus Genome. Genes 12 30477134
2014 Full-genome identification and characterization of NBS-encoding disease resistance genes in wheat. Molecular genetics and genomics : MGG 12 25231182
2024 Promises and challenges of genomic newborn screening (NBS) - lessons from public health NBS programs. Pediatric research 11 39516573
2023 Indispensable biomolecules for plant defense against pathogens: NBS-LRR and "nitrogen pool" alkaloids. Plant science : an international journal of experimental plant biology 11 37268110
2022 The Cassava NBS-LRR Genes Confer Resistance to Cassava Bacterial Blight. Frontiers in plant science 11 35178059
2022 NBS-LRR-WRKY genes and protease inhibitors (PIs) seem essential for cowpea resistance to root-knot nematode. Journal of proteomics 11 35351660
2021 Genome-wide Identification and Evolutionary Analysis of NBS-LRR Genes From Secale cereale. Frontiers in genetics 11 34858486
2017 The Ambystoma tigrinum virus (ATV) RNase III gene can modulate host PKR activation and interferon production. Virology 11 28844332
2016 Tracking ancestral lineages and recent expansions of NBS-LRR genes in angiosperms. Plant signaling & behavior 11 27348446
2009 A systematic proteomic study of irradiated DNA repair deficient Nbn-mice. PloS one 11 19412544
2022 NBN, RAD51 and XRCC3 Polymorphisms as Potential Predictive Biomarkers of Adjuvant Radiotherapy Toxicity in Early HER2-Positive Breast Cancer. Cancers 10 36139526
2019 DNA repair functional analyses of NBN hypomorphic variants associated with NBN-related infertility. Human mutation 10 31729086
2010 High prevalence of the NBN gene mutation c.657-661del5 in Southeast Germany. Journal of applied genetics 10 20453309

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