Affinage

Showing ATRX — RAD54 is a alias.

ATRX

Transcriptional regulator ATRX · UniProt P46100

Length
2492 aa
Mass
282.6 kDa
Annotated
2026-06-09
100 papers in source corpus 28 papers cited in narrative 28 extracted findings
Cross-family judge vs UniProt: Affinage preferred faithfulness: 8/8 claims corpus-supported (100%)

Mechanistic narrative

Synthesis pass · prose summary of the discoveries below

ATRX is an ATP-dependent SWI/SNF-family chromatin regulator that maintains heterochromatin integrity at repetitive and structurally challenging genomic loci, with loss of its activities driving genomic instability (PMID:18227278, PMID:34162889). Through its zinc-finger domain it binds double-stranded DNA, and disease-causing ATR-X mutations in this domain abolish DNA binding and impair nuclear localization (PMID:11015451). Acting with its partner DAXX, ATRX deposits histone variant H3.3 to establish and maintain repressed chromatin states at rDNA repeats, p53 response elements, and viral genomes, where it restricts chromatin accessibility rather than initiating histone deposition (PMID:29669917, PMID:33909709, PMID:36028493). ATRX is also a high-affinity RNA-binding protein with RNA-binding regions distinct from its PHD and helicase domains; it directly binds RepA/Xist RNA to load PRC2 in cis and is antagonized at telomeres by TERRA RNA, which competes for ATRX binding and modulates its genome-wide occupancy (PMID:25417162, PMID:32376827, PMID:28666128). A central activity is the resolution of G-quadruplex structures: ATRX associates with the MCM replication complex and uses its helicase and H3.3-chaperone functions to suppress G4 accumulation at newly synthesized DNA upstream of ESET-mediated heterochromatin formation, and recruits DNA methyltransferases to G4-containing CpG islands to silence target genes (PMID:34162889, PMID:29785027). In DNA repair, ATRX promotes a crossover-prone homologous-recombination subpathway downstream of Rad51 removal, interacting with PCNA and RFC-1 to drive repair synthesis that outcompetes RECQ5-dependent SDSA and generates sister-chromatid exchanges resolved by MUS81/GEN1 (PMID:29937341, PMID:33431668). ATRX additionally ensures accurate mitotic and meiotic chromosome segregation (PMID:18227278, PMID:22918800), suppresses alternative lengthening of telomeres (PMID:26001292), and regulates checkpoint and immune gene expression including CHEK1 and the cGAS/STING pathway (PMID:35021084, PMID:37200088). Mutations in the ATRX DNA-binding domain underlie the ATR-X intellectual disability syndrome (PMID:11015451).

Mechanistic history

Synthesis pass · year-by-year structured walk · 12 steps
  1. 1998 Low

    Established an early physical link between ATRX and the Polycomb machinery, raising the hypothesis that ATRX regulates transcription through chromatin remodeling with PRC2.

    Evidence Yeast two-hybrid against the EZH2 SET domain

    PMID:9499421

    Open questions at the time
    • Single yeast two-hybrid without biochemical validation of a direct interaction
    • No functional consequence demonstrated
    • No cellular localization of the interaction
  2. 2000 Medium

    Defined the zinc-finger domain as ATRX's DNA-binding module and connected its mutation to the ATR-X disease phenotype.

    Evidence In vitro DNA binding assays plus immunocytochemistry and western blot in ATR-X patient cells

    PMID:11015451

    Open questions at the time
    • Sequence specificity of DNA binding not defined
    • Link between DNA-binding loss and chromatin function not established
    • Single lab
  3. 2008 High

    Demonstrated ATRX is required for faithful mitosis, extending its role from transcription to chromosome segregation.

    Evidence Live imaging, RNAi, and a mouse neuroprogenitor genetic model with immunofluorescence

    PMID:18227278

    Open questions at the time
    • Molecular basis for cohesion/congression defect not resolved
    • Direct cohesion machinery partners not identified
  4. 2010 High

    Connected ATRX biochemically and genetically to HP1-dependent pericentric heterochromatin assembly, using invertebrate models.

    Evidence Co-IP, in vitro ATPase assay, immunolocalization, and PEV genetics in Drosophila (with related C. elegans epistasis)

    PMID:15328017 PMID:15649460 PMID:19706533 PMID:20154359

    Open questions at the time
    • Direct extrapolation to human ATRX isoforms not shown
    • Mechanism of HP1a stimulation of ATPase not detailed
  5. 2014 High

    Revealed ATRX as a high-affinity RNA-binding protein that directs PRC2 loading onto Xist RNA, defining a non-canonical RNA-guided chromatin function.

    Evidence Unbiased proteomics, RNA-IP, ChIP-seq and epigenomic profiling with ATRX KO/KD

    PMID:25417162

    Open questions at the time
    • RNA-binding domain not yet mapped at this stage
    • How RNA binding integrates with DNA/H3.3 functions unclear
  6. 2015 High

    Provided direct functional evidence that ATRX represses the ALT telomere-maintenance pathway, but only in cooperation with other alterations.

    Evidence Transient ATRX re-expression rescue in ALT-positive cells with C-circle/APB assays

    PMID:26001292

    Open questions at the time
    • Identity of cooperating alterations needed for ALT not defined
    • Mechanistic link to H3.3/telomeric chromatin not fully resolved
  7. 2017 High

    Defined ATRX's role in heterochromatin-dependent senescence and identified TERRA as an RNA competitor that restricts ATRX from telomeres.

    Evidence ChIRP-seq/RIP/ATRX ChIP and TERRA depletion (telomere study); ChIP, KD/KO and senescence assays with HRAS repression

    PMID:28666128 PMID:28855512

    Open questions at the time
    • Structural basis of RNA-vs-DNA competition not solved
    • Direct connection between senescence foci and ALT suppression unclear
  8. 2018 High

    Established ATRX's role in homologous recombination repair synthesis and extended its chromatin/H3.3 functions to rDNA, imprinted G4 loci, and viral chromatin.

    Evidence Co-IP (PCNA/RFC-1), SCE and repair-synthesis assays; H3.3 ChIP at rDNA; G4/CpG ChIP and DNMT recruitment in mouse brain; ATRX/DAXX knockdown in EBV and HSV systems

    PMID:22102817 PMID:29669917 PMID:29785027 PMID:29937341 PMID:30465651

    Open questions at the time
    • Whether HR, rDNA, and viral roles share one molecular mechanism not established
    • NHEJ versus HR balance (cf. PMID 26936505) not reconciled
  9. 2020 High

    Mapped ATRX's RNA-binding regions to the N-terminus, distinct from PHD and helicase domains, and showed RNA binding shapes ATRX chromatin distribution and PRC2 localization.

    Evidence In vitro RNA binding, RIP, and ChIP-seq of an ATRX-deltaRBR deletion mutant with PRC2 ChIP-seq

    PMID:32376827 PMID:36440760

    Open questions at the time
    • Sequence/structure determinants of RBR specificity not resolved
    • Interplay of RBR with helicase activity not defined
  10. 2021 High

    Integrated ATRX into replication-coupled G-quadruplex resolution and defined a crossover-prone HR subpathway distinct from SDSA.

    Evidence Co-IP with MCM, ATRX domain mutants and ESET epistasis with G4 imaging; ATRX/RECQ5/PCNA epistasis with SCE and MUS81/GEN1 dependence; HSV ATAC-seq

    PMID:33431668 PMID:33909709 PMID:34162889

    Open questions at the time
    • Order of events linking G4 unwinding, H3.3 deposition, and ESET methylation not fully resolved
    • Structure of the ATRX-pathway HR joint molecules not determined
  11. 2022 Medium

    Connected ATRX/DAXX-dependent H3.3 deposition to p53 chromatin access and checkpoint gene control, linking chromatin function to the DNA damage response.

    Evidence p53/H3.3/gammaH2AX ChIP-seq and ATAC-seq with ATRX/DAXX KO; ATRX ChIP and CRISPR KO with CHEK1/cell-cycle readouts

    PMID:35021084 PMID:36028493

    Open questions at the time
    • Direct versus indirect effect on p53 binding not separated
    • Single lab for each finding
  12. 2023 Medium

    Linked ATRX loss to suppression of cGAS/STING innate immune signaling, connecting its chromatin role to tumor immune evasion.

    Evidence Primary Atrx-deleted mouse sarcoma model with gene expression and immune profiling

    PMID:37200088

    Open questions at the time
    • Molecular step at which ATRX modulates cGAS/STING not pinpointed
    • Whether effect is chromatin-mediated not established

Open questions

Synthesis pass · forward-looking unresolved questions
  • How ATRX's distinct activities — DNA/G4 binding, RNA binding, H3.3 deposition, helicase remodeling, and HR repair synthesis — are coordinated into a single integrated mechanism at specific loci remains unresolved.
  • No unifying structural/biochemical model integrating ATRX domains
  • Locus-selectivity determinants between functions unknown
  • Reconciliation of NHEJ versus HR roles incomplete

Mechanism profile

Synthesis pass · controlled-vocabulary classification · explore literature graph →
Molecular activity
GO:0003677 DNA binding 4 GO:0140096 catalytic activity, acting on a protein 4 GO:0003723 RNA binding 3 GO:0140657 ATP-dependent activity 2 GO:0042393 histone binding 1
Localization
GO:0005694 chromosome 3 GO:0005634 nucleus 2 GO:0005730 nucleolus 1
Pathway
R-HSA-4839726 Chromatin organization 4 R-HSA-1640170 Cell Cycle 3 R-HSA-73894 DNA Repair 3 R-HSA-74160 Gene expression (Transcription) 3
Partners
DAXXEZH2HP1AMCMPCNARFC1
Complex memberships
ATRX/DAXX H3.3 chaperone complexPRC2 (functional association)

Evidence

Reading pass · 28 per-paper findings extracted from the source corpus
Year Finding Method Journal Conf PMIDs
1998 The XNP/ATRX protein interacts specifically with the SET domain of human EZH2, as demonstrated by yeast two-hybrid analysis, suggesting ATRX regulates gene transcription through chromatin remodeling in association with PRC2 components. Yeast two-hybrid assay Human molecular genetics Low 9499421
2000 The zinc finger domain of XNP/ATRX mediates double-stranded DNA binding in vitro, and disease-causing mutations in this domain severely reduce DNA binding capacity; additionally, ATR-X patient cells show altered or absent XNP/ATRX protein expression and impaired nuclear localization. In vitro DNA binding assays, immunocytochemistry, western blot with patient-derived cells and monoclonal antibodies Journal of medical genetics Medium 11015451
2008 ATRX is required for normal mitotic progression in human cultured cells and mouse neuroprogenitors; loss of ATRX causes defective sister chromatid cohesion and chromosome congression at the metaphase plate, as shown by live cell imaging and analysis of embryonic mouse brain neuroprogenitors. Live cell imaging, RNAi-mediated depletion, mouse genetic model, immunofluorescence The Journal of cell biology High 18227278
2009 Drosophila XNP (ATRX ortholog) localizes to active genes and a decondensed satellite DNA focus near heterochromatin on the X chromosome, corresponding to sites of ongoing nucleosome replacement; XNP modulates nucleosome dynamics at these sites to limit chromatin accessibility and contributes to heterochromatic gene silencing. Immunolocalization, position-effect variegation assays, overexpression in Drosophila Proceedings of the National Academy of Sciences of the United States of America Medium 19706533
2010 Drosophila ATRX forms a complex with HP1a; the ATRX185 isoform but not ATRX125 is concentrated in pericentric beta-heterochromatin of the X chromosome, HP1a strongly stimulates ATRX185 biochemical activities in vitro, and ATRX185 is required for HP1a deposition in pericentric heterochromatin. Loss-of-function ATRX alleles suppress position effect variegation. Biochemical fractionation, co-immunoprecipitation, in vitro ATPase assay, immunolocalization, Drosophila genetics (PEV suppression) The Journal of biological chemistry High 20154359
2011 C. elegans xnp-1 (ATRX ortholog) functions together with lin-35/Rb, hpl-2/HP1, and the NuRD complex during development; double mutants show larval arrest with cessation of growth; xnp-1 and lin-35 jointly control transgene silencing via chromatin remodeling. C. elegans genetic epistasis, RNAi, transgene silencing assay Developmental biology Medium 15649460
2012 ATRX and DAXX are required for transcriptional repression and chromatin assembly at a CMV-promoter transgene array; the array is refractory to activation in ATRX/DAXX-expressing cells but can be robustly activated in ATRX-negative U2OS cells. HSV-1 ICP0 depletes ATRX and DAXX from the array upon activation, and histone H3.3 is recruited but not incorporated into chromatin during activation, indicating ATRX/DAXX maintain a repressed chromatin environment through H3.3 deposition. Single-cell live imaging, inducible transgene array, ATRX-negative cell line comparison, ICP0 expression, histone H3.3 recruitment assay Journal of cell science Medium 22976303
2014 ATRX functions as a high-affinity RNA-binding protein that directly interacts with RepA/Xist RNA to promote loading of PRC2 in vivo. Without ATRX, PRC2 cannot load onto Xist RNA or spread in cis along the X chromosome. Genome-wide, loss of ATRX leads to spatial redistribution of PRC2 and derepression of Polycomb-responsive genes. Unbiased proteomics (mass spectrometry), RNA immunoprecipitation, ChIP-seq, epigenomic profiling, ATRX knockout/knockdown Cell High 25417162
2015 Transient ATRX expression in ALT-positive/ATRX-negative cells directly represses ALT activity, providing functional evidence that ATRX represses the ALT mechanism. ATRX loss alone in mortal or telomerase-positive cells is insufficient to activate ALT; it requires cooperation with additional genetic/epigenetic alterations. ATRX knockout/knockdown, transient ATRX re-expression in ALT-positive cells, ALT assays (C-circle, APB formation) Oncotarget High 26001292
2016 ATRX deficiency impairs nonhomologous end joining (NHEJ) DNA repair in glioma cells and increases sensitivity to DNA-damaging agents that induce double-strand breaks, establishing a role for ATRX in NHEJ. ATRX-deficient mouse glioma model, NHEJ repair assays, DNA damage sensitivity assays Science translational medicine Medium 26936505
2017 TERRA RNA competes with telomeric DNA for ATRX binding, suppresses ATRX localization to telomeres, and ensures telomeric stability. TERRA and ATRX are functionally antagonistic at shared target genes: TERRA activates while ATRX represses gene expression at these loci. Genomic (ChIRP-seq) and proteomic approaches, RNA immunoprecipitation, ATRX ChIP, TERRA depletion, telomere stability assays Cell High 28666128
2017 ATRX accumulates in nuclear foci during therapy-induced senescence in a manner dependent on its ability to interact with H3K9me3 histone and HP1; ATRX is required for therapy-induced senescence across multiple transformed cell types, and loss of ATRX in senescent cells destabilizes senescence-associated heterochromatic foci. Additionally, ATRX binds to and suppresses expression from the HRAS locus, and repression of HRAS is sufficient to promote quiescent-to-senescent transition. ATRX depletion/knockout, live cell imaging of nuclear foci, ChIP for H3K9me3/HP1, HRAS locus ChIP, gene expression analysis, senescence assays Nature communications High 28855512
2018 ATRX operates downstream of the Rad51 removal step in homologous recombination and interacts with PCNA and RFC-1 to promote DNA repair synthesis during HR. ATRX depletion abolishes DNA repair synthesis and sister chromatid exchange at exogenously induced DSBs. ATRX and DAXX together deposit histone H3.3 during DNA repair synthesis, indicating ATRX facilitates chromatin reconstitution required for extended repair synthesis. Co-immunoprecipitation (ATRX-PCNA, ATRX-RFC-1), ATRX/DAXX/H3.3 siRNA depletion, SCE assay, DNA repair synthesis assay, epistasis analysis Molecular cell High 29937341
2018 ATRX forms a complex with EZH2, and this ATRX/EZH2 complex epigenetically regulates FADD/PARP1 axis in glioma: ATRX downregulates FADD expression via H3K27me3 enrichment at the FADD locus in an EZH2-dependent manner, which stabilizes PARP1 protein, contributing to TMZ resistance. CRISPR-Cas9 ATRX knockout, ChIP-seq (H3K27me3), co-immunoprecipitation (ATRX/EZH2), gene expression analysis, in vitro and in vivo tumor assays Theranostics Medium 32194873
2018 ATRX depletion in mouse ES cells leads to loss of rDNA copy number through disruption of H3.3 deposition and failure of heterochromatin formation at rDNA repeats; ATRX-depleted cells show reduced ribosomal RNA transcription and increased sensitivity to Pol I inhibitor CX5461. ATRX depletion in mouse ES cells, H3.3 ChIP, rDNA copy number analysis, rRNA transcription assay, drug sensitivity assay Proceedings of the National Academy of Sciences of the United States of America Medium 29669917
2018 ATRX binds to G-quadruplexes in CpG islands of the imprinted Xlr3b gene in mouse brain and regulates its expression by recruiting DNA methyltransferases; ATRX mutation leads to aberrant upregulation of Xlr3b, which inhibits dendritic mRNA transport and impairs synaptic function. ChIP (ATRX at G4/CpG islands), G4 binding assay, DNA methyltransferase recruitment assay, dendritic transport assay, ATR-X mouse model Nature medicine Medium 29785027
2018 The EBV tegument protein BNRF1 interacts with host Daxx at PML nuclear bodies and disrupts the Daxx-ATRX chromatin remodeling complex; knockdown of DAXX and ATRX induces EBV reactivation from latency, demonstrating that the Daxx-ATRX complex regulates viral chromatin and suppresses EBV lytic reactivation. Co-immunoprecipitation (BNRF1-Daxx), ATRX/Daxx knockdown, RT-PCR for viral gene expression, EBV reactivation assay PLoS pathogens Medium 22102817
2018 ATRX colocalizes with herpes simplex virus DNA within 15 minutes of nuclear entry, and although initial viral heterochromatin formation is ATRX-independent, ATRX is specifically required for maintaining viral heterochromatin stability from 4 to 8 hours post-infection during transcriptional stress. Bioorthogonal genome labeling, ATRX depletion (fibroblasts), HSV infection assay, viral mRNA quantification, viral DNA accumulation assay, inhibition of transcription eLife High 30465651
2020 TERRA modulates ATRX occupancy on repetitive sequences and over genes, and maintains DNA G-quadruplex structures at TERRA target and non-target sites. TERRA prevents ATRX from binding to subtelomeric regions and represses H3K9me3 formation; knockdown of TERRA reduces DNA G4 signals whereas ATRX silencing elevates G4 formation, indicating ATRX and TERRA oppositely regulate G4 structures. G4 ChIP-seq, ATRX ChIP-seq, TERRA depletion, ATRX knockdown, H3K9me3 ChIP Nucleic acids research Medium 36440760
2020 ATRX has RNA binding regions (RBRs) distinct from its PHD and helicase domains; deletion of the major ATRX RBR in the N-terminal region disrupts ATRX interactions with RNA in vitro and in vivo, alters its chromatin binding properties, results in redistribution of ATRX on chromatin, and affects PRC2 localization at a subset of polycomb target genes. RNA immunoprecipitation, in vitro RNA binding assay, ChIP-seq (ATRX-ΔRBR), ATRX deletion mutant analysis, PRC2 ChIP-seq Nature communications High 32376827
2021 ATRX associates with MCM replication complex subunits; loss of ATRX leads to G-quadruplex structure accumulation at newly synthesized DNA. Both the helicase domain and H3.3 chaperone function of ATRX are required to protect cells from G4-induced replicative stress, and these activities are upstream of heterochromatin formation mediated by ESET histone methyltransferase. Co-immunoprecipitation (ATRX-MCM), ATRX domain mutants, G4 immunofluorescence at newly synthesized DNA, ESET epistasis, ATRX knockout Nature communications High 34162889
2021 ATRX limits accessibility of histone H3-loaded HSV genomes to reduce viral DNA accessibility for transcription; ATRX/DAXX complex is unique among nuclear H3 chaperones in restricting ICP0-null HSV infection. ATRX is not required for initial H3 deposition on viral DNA but reduces viral DNA accessibility as shown by ATAC-seq and enhanced nucleosome-like structure accumulation. Systematic depletion of nuclear H3 chaperones, ChIP-seq (total H3), ATAC-seq, ATRX-KO fibroblasts, ICP0-null HSV infection assay PLoS pathogens High 33909709
2021 ATRX-dependent HR outcompetes RECQ5-dependent SDSA for the repair of most two-ended DSBs in human cells, frequently forming sister chromatid exchanges (crossovers). Subpathway choice depends on interaction of both ATRX and RECQ5 with PCNA. ATRX-pathway HR intermediates require MUS81 and GEN1 (but not BLM) for resolution, suggesting formation of joint molecules distinct from classical Holliday junctions. ATRX/RECQ5/PCNA knockouts and epistasis, SCE assay, MUS81/GEN1 knockdown, HR reporter assay, ultra-fine bridge analysis Proceedings of the National Academy of Sciences of the United States of America High 33431668
2022 Loss of ATRX or DAXX leads to genome-wide reduction in p53 DNA binding and loss of chromatin accessibility at p53 response elements, associated with depletion of histone H3.3 and accumulation of γH2AX at p53 sites including subtelomeres, indicating ATRX/DAXX-dependent H3.3 deposition is required for p53 chromatin access and DNA damage response. ChIP-seq (p53), ATAC-seq, H3.3 and γH2AX ChIP-seq, DAXX/ATRX knockout Nature communications Medium 36028493
2022 ATRX binds regulatory elements of cell-cycle phase transition genes in GBM cells; ATRX loss leads to marked reduction in CHEK1 (Checkpoint Kinase 1) expression, causing early release of G2/M entry after irradiation and enhanced ATM activation. ATRX ChIP, ATRX loss-of-function (CRISPR), CHEK1 expression analysis, cell cycle assay post-irradiation, ATM activation assay Cell reports Medium 35021084
2023 Atrx deletion results in downregulation of the cGAS/STING innate immune signaling pathway at multiple points without transcriptional downregulation or mutations in pathway components; Atrx-deleted sarcomas show reduced adaptive immune response and impaired cGAS/STING signaling. Primary mouse sarcoma model (Atrx deletion), gene expression analysis, in vivo tumor treatment assays, immune profiling The Journal of clinical investigation Medium 37200088
2004 C. elegans xnp-1 (ATRX ortholog) and lin-35/Rb function redundantly in somatic gonad development; xnp-1;lin-35 double mutants are sterile with severe defects in sheath and spermatheca lineages, establishing a functional genetic interaction between ATRX and Rb family members. C. elegans genetic epistasis, double-mutant analysis, GFP reporter for xnp-1 expression Developmental biology Medium 15328017
2012 In mammalian oocytes, ATRX binds to centromeric heterochromatin and is required for accurate chromosome segregation during meiosis; loss of ATRX induces abnormal meiotic chromosome morphology, reduces histone H3 phosphorylation at centromeres, and promotes high-incidence aneuploidy associated with severely reduced fertility. ATRX loss-of-function in mouse oocytes, immunofluorescence, chromosome spread analysis, fertility assay Results and problems in cell differentiation Medium 22918800

Source papers

Stage 0 corpus · 100 papers · ranked by NIH iCite citations
Year Title Journal Citations PMID
1998 Catalysis of homologous DNA pairing by yeast Rad51 and Rad54 proteins. Nature 361 9590697
2016 ATRX loss promotes tumor growth and impairs nonhomologous end joining DNA repair in glioma. Science translational medicine 224 26936505
2012 Whole-exome sequencing identifies ATRX mutation as a key molecular determinant in lower-grade glioma. Oncotarget 221 23104868
2017 TERRA RNA Antagonizes ATRX and Protects Telomeres. Cell 218 28666128
2006 Rad54: the Swiss Army knife of homologous recombination? Nucleic acids research 206 16935872
2000 Superhelicity-driven homologous DNA pairing by yeast recombination factors Rad51 and Rad54. Molecular cell 191 11030336
2018 ATRX, DAXX or MEN1 mutant pancreatic neuroendocrine tumors are a distinct alpha-cell signature subgroup. Nature communications 178 30315258
2014 ATRX directs binding of PRC2 to Xist RNA and Polycomb targets. Cell 172 25417162
2000 Molecular-clinical spectrum of the ATR-X syndrome. American journal of medical genetics 162 11449489
2010 Rad54, the motor of homologous recombination. DNA repair 142 20089461
2015 ATRX represses alternative lengthening of telomeres. Oncotarget 138 26001292
1996 Human and mouse homologs of the Saccharomyces cerevisiae RAD54 DNA repair gene: evidence for functional conservation. Current biology : CB 136 8805304
2008 Loss of ATRX leads to chromosome cohesion and congression defects. The Journal of cell biology 134 18227278
2006 Visualization of Rad54, a chromatin remodeling protein, translocating on single DNA molecules. Molecular cell 134 16818238
1998 Specific interaction between the XNP/ATR-X gene product and the SET domain of the human EZH2 protein. Human molecular genetics 123 9499421
2011 Functions of the Snf2/Swi2 family Rad54 motor protein in homologous recombination. Biochimica et biophysica acta 122 21704205
2018 ATRX Promotes DNA Repair Synthesis and Sister Chromatid Exchange during Homologous Recombination. Molecular cell 121 29937341
2002 Homologous DNA pairing by human recombination factors Rad51 and Rad54. The Journal of biological chemistry 118 12205100
1987 Regulation of RAD54- and RAD52-lacZ gene fusions in Saccharomyces cerevisiae in response to DNA damage. Molecular and cellular biology 118 3550429
2018 Mutant ATRX: uncovering a new therapeutic target for glioma. Expert opinion on therapeutic targets 117 29889582
2011 EBV tegument protein BNRF1 disrupts DAXX-ATRX to activate viral early gene transcription. PLoS pathogens 116 22102817
2002 RAD18 and RAD54 cooperatively contribute to maintenance of genomic stability in vertebrate cells. The EMBO journal 113 12374756
1998 The human RAD54 recombinational DNA repair protein is a double-stranded DNA-dependent ATPase. The Journal of biological chemistry 109 9774452
2015 IDH mutation, 1p19q codeletion and ATRX loss in WHO grade II gliomas. Oncotarget 108 26210286
2004 Rad54 and DNA Ligase IV cooperate to maintain mammalian chromatid stability. Genes & development 102 15175260
2013 The chromatin remodeller ATRX: a repeat offender in human disease. Trends in biochemical sciences 98 23916100
2020 ATRX/EZH2 complex epigenetically regulates FADD/PARP1 axis, contributing to TMZ resistance in glioma. Theranostics 94 32194873
2020 MYCN amplification and ATRX mutations are incompatible in neuroblastoma. Nature communications 91 32060267
2021 ATRX promotes heterochromatin formation to protect cells from G-quadruplex DNA-mediated stress. Nature communications 85 34162889
2017 The Role of ATRX in Glioma Biology. Frontiers in oncology 84 29034211
2023 ATRX, a guardian of chromatin. Trends in genetics : TIG 81 36894374
2020 Rad54 Drives ATP Hydrolysis-Dependent DNA Sequence Alignment during Homologous Recombination. Cell 81 32502392
2017 New Molecular Considerations for Glioma: IDH, ATRX, BRAF, TERT, H3 K27M. Current neurology and neuroscience reports 81 28271343
2016 Nek1 Regulates Rad54 to Orchestrate Homologous Recombination and Replication Fork Stability. Molecular cell 81 27264870
2001 Rad54 protein stimulates the postsynaptic phase of Rad51 protein-mediated DNA strand exchange. Proceedings of the National Academy of Sciences of the United States of America 80 11459988
2018 Ribosomal DNA copy loss and repeat instability in ATRX-mutated cancers. Proceedings of the National Academy of Sciences of the United States of America 79 29669917
2011 ATP-dependent and independent functions of Rad54 in genome maintenance. The Journal of cell biology 78 21357745
2016 The Role of ATRX in the Alternative Lengthening of Telomeres (ALT) Phenotype. Genes 73 27657132
2017 ATRX is a regulator of therapy induced senescence in human cells. Nature communications 72 28855512
2021 Corticotroph Aggressive Pituitary Tumors and Carcinomas Frequently Harbor ATRX Mutations. The Journal of clinical endocrinology and metabolism 71 33106857
2018 Targeting G-quadruplex DNA as cognitive function therapy for ATR-X syndrome. Nature medicine 68 29785027
2015 RAD54 family translocases counter genotoxic effects of RAD51 in human tumor cells. Nucleic acids research 63 25765654
2003 Acquired somatic ATRX mutations in myelodysplastic syndrome associated with alpha thalassemia (ATMDS) convey a more severe hematologic phenotype than germline ATRX mutations. Blood 63 14592816
2020 The bromodomain containing protein BRD-9 orchestrates RAD51-RAD54 complex formation and regulates homologous recombination-mediated repair. Nature communications 62 32457312
2018 ATRX promotes maintenance of herpes simplex virus heterochromatin during chromatin stress. eLife 61 30465651
2002 Analysis of mouse Rad54 expression and its implications for homologous recombination. DNA repair 61 12531026
1997 Determination of the genomic structure of the XNP/ATRX gene encoding a potential zinc finger helicase. Genomics 57 9244431
2006 Terminal association of Rad54 protein with the Rad51-dsDNA filament. Proceedings of the National Academy of Sciences of the United States of America 56 16785421
2022 ATRX loss in glioma results in dysregulation of cell-cycle phase transition and ATM inhibitor radio-sensitization. Cell reports 55 35021084
2016 Immunohistochemical Analysis of ATRX, IDH1 and p53 in Glioblastoma and Their Correlations with Patient Survival. Journal of Korean medical science 53 27478330
2015 Emerging roles of ATRX in cancer. Epigenomics 53 26646632
1996 A novel mutation in the putative DNA helicase XH2 is responsible for male-to-female sex reversal associated with an atypical form of the ATR-X syndrome. American journal of human genetics 53 8651295
1996 Splicing mutation in the ATR-X gene can lead to a dysmorphic mental retardation phenotype without alpha-thalassemia. American journal of human genetics 52 8644709
2020 Correlation between IDH, ATRX, and TERT promoter mutations in glioma. Brain tumor pathology 51 32227259
2007 Metabolic syndrome and C-reactive protein in the general population: JMS Cohort Study. Circulation journal : official journal of the Japanese Circulation Society 51 17186974
2012 Single-cell analysis of Daxx and ATRX-dependent transcriptional repression. Journal of cell science 46 22976303
2011 Role of ATRX in chromatin structure and function: implications for chromosome instability and human disease. Reproduction (Cambridge, England) 46 21653732
2021 ATRX regulates glial identity and the tumor microenvironment in IDH-mutant glioma. Genome biology 44 34763709
2000 ATR-X mutations cause impaired nuclear location and altered DNA binding properties of the XNP/ATR-X protein. Journal of medical genetics 43 11015451
2022 ATRX loss promotes immunosuppressive mechanisms in IDH1 mutant glioma. Neuro-oncology 42 34951647
2022 DAXX-ATRX regulation of p53 chromatin binding and DNA damage response. Nature communications 42 36028493
2014 Altered global histone-trimethylation code and H3F3A-ATRX mutation in pediatric GBM. Journal of neuro-oncology 42 25479829
2019 In vitro role of Rad54 in Rad51-ssDNA filament-dependent homology search and synaptic complexes formation. Nature communications 41 31492866
2021 Loss of ATRX confers DNA repair defects and PARP inhibitor sensitivity. Translational oncology 40 34118569
2013 ATRX and the replication of structured DNA. Current opinion in genetics & development 40 23453691
2005 Mutation in the 5' alternatively spliced region of the XNP/ATR-X gene causes Chudley-Lowry syndrome. European journal of human genetics : EJHG 37 15508018
2019 Targeting Telomerase and ATRX/DAXX Inducing Tumor Senescence and Apoptosis in the Malignant Glioma. International journal of molecular sciences 36 30625996
2009 The XNP remodeler targets dynamic chromatin in Drosophila. Proceedings of the National Academy of Sciences of the United States of America 35 19706533
2002 Expanding phenotype of XNP mutations: mild to moderate mental retardation. American journal of medical genetics 35 12116232
2022 TOP3A amplification and ATRX inactivation are mutually exclusive events in pediatric osteosarcomas using ALT. EMBO molecular medicine 34 35920001
2008 Human Rad54 protein stimulates human Mus81-Eme1 endonuclease. Proceedings of the National Academy of Sciences of the United States of America 34 19017809
2023 The Chromatin Remodeler ATRX: Role and Mechanism in Biology and Cancer. Cancers 33 37190157
2021 The Multiple Facets of ATRX Protein. Cancers 33 34062956
2007 Interactions of human rad54 protein with branched DNA molecules. The Journal of biological chemistry 32 17545145
2004 lin-35/Rb and xnp-1/ATR-X function redundantly to control somatic gonad development in C. elegans. Developmental biology 32 15328017
2017 RAD54 forms DNA repair foci in response to DNA damage in living plant cells. The Plant journal : for cell and molecular biology 31 28155243
2010 Protein complex of Drosophila ATRX/XNP and HP1a is required for the formation of pericentric beta-heterochromatin in vivo. The Journal of biological chemistry 31 20154359
2018 Loss of ATRX/DAXX expression and alternative lengthening of telomeres in uterine leiomyomas. Cancer 29 30423196
2020 Disruption of ATRX-RNA interactions uncovers roles in ATRX localization and PRC2 function. Nature communications 28 32376827
2011 Protein-DNA interactions in high speed AFM: single molecule diffusion analysis of human RAD54. Integrative biology : quantitative biosciences from nano to macro 28 21986699
2005 XNP-1/ATR-X acts with RB, HP1 and the NuRD complex during larval development in C. elegans. Developmental biology 28 15649460
2022 Mutational spectrum of ATRX aberrations in neuroblastoma and associated patient and tumor characteristics. Cancer science 27 35384159
2021 ATRX and RECQ5 define distinct homologous recombination subpathways. Proceedings of the National Academy of Sciences of the United States of America 27 33431668
2020 NUCKS1 promotes RAD54 activity in homologous recombination DNA repair. The Journal of cell biology 27 32876692
2018 Alternative lengthening of telomeres phenotype and loss of ATRX expression in sarcomas. Oncology letters 27 29725455
2024 Interplay between ATRX and IDH1 mutations governs innate immune responses in diffuse gliomas. Nature communications 26 38272925
2022 TERRA regulates DNA G-quadruplex formation and ATRX recruitment to chromatin. Nucleic acids research 26 36440760
2011 ATRX in chromatin assembly and genome architecture during development and disease. Biochemistry and cell biology = Biochimie et biologie cellulaire 26 21851155
2003 Genome instability in rad54 mutants of Saccharomyces cerevisiae. Nucleic acids research 26 12560498
2021 ATRX limits the accessibility of histone H3-occupied HSV genomes during lytic infection. PLoS pathogens 25 33909709
1996 A point mutation in the XNP gene, associated with an ATR-X phenotype without alpha-thalassemia. European journal of human genetics : EJHG 25 9043863
2023 Atrx deletion impairs CGAS/STING signaling and increases sarcoma response to radiation and oncolytic herpesvirus. The Journal of clinical investigation 24 37200088
2023 TLK1-mediated RAD54 phosphorylation spatio-temporally regulates Homologous Recombination Repair. Nucleic acids research 24 37439356
2004 ATRX and sex differentiation. Trends in endocrinology and metabolism: TEM 24 15350606
2008 Rad51 protein stimulates the branch migration activity of Rad54 protein. The Journal of biological chemistry 23 18617519
2021 ATR-X syndrome: genetics, clinical spectrum, and management. Human genetics 21 34524523
2020 Rdh54/Tid1 inhibits Rad51-Rad54-mediated D-loop formation and limits D-loop length. eLife 21 33185188
2012 Chromatin structure and ATRX function in mouse oocytes. Results and problems in cell differentiation 21 22918800
2004 Lig4 and rad54 are required for repair of DNA double-strand breaks induced by P-element excision in Drosophila. Genetics 21 15545651
2017 Clinicopathological analysis of ATRX, DAXX and NOTCH receptor expression in angiosarcomas. Histopathology 20 28796347

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