Affinage

BCOR

BCL-6 corepressor · UniProt Q6W2J9

Length
1755 aa
Mass
192.2 kDa
Annotated
2026-06-09
100 papers in source corpus 23 papers cited in narrative 23 extracted findings
Cross-family judge vs UniProt: Affinage preferred faithfulness: 7/7 claims corpus-supported (100%)

Mechanistic narrative

Synthesis pass · prose summary of the discoveries below

BCOR is a transcriptional corepressor that nucleates a non-canonical Polycomb repressive complex (PRC1.1) to silence developmental and oncogenic gene programs across hematopoietic, neural, mesenchymal, and epithelial lineages (PMID:10898795, PMID:26847029, PMID:29337181). It was first defined as a selective binding partner of the BCL6 POZ domain — an interaction mutually exclusive with BCL6's association with SMRT/N-CoR — that potentiates BCL6-mediated repression and recruits class I/II HDACs (PMID:10898795). BCOR assembles PRC1.1 by linking the RING1/RNF2-PCGF1 catalytic core, which monoubiquitinates histone H2A at K119, to the chromatin-targeting subunit KDM2B/FBXL10 (a JmjC H3K36 demethylase) together with RYBP, NSPC1, and SKP1; these components and H2AK119ub co-occupy BCL6 target genes (PMID:16943429). The BCOR C-terminal PUFD domain mediates this assembly: its termini are disordered and become ordered upon binding PCGF1, enabling stable KDM2B association, and internal tandem duplications mapping to the PUFD termini disrupt PRC1.1 assembly (PMID:32628469). Through deposition of H2AK119ub, BCOR-PRC1.1 represses HOX genes, Myc, Gli1/Gli2, Igf2, and androgen-repressed loci, and loss of BCOR or its truncation reduces RING1B levels and H2AK119ub at these targets, driving their derepression (PMID:26847029, PMID:28827447, PMID:32820036, PMID:31925334). BCOR functions as a tumor suppressor: disruptive mutations or C-terminal truncations that unlink the enzymatic core from the chromatin-targeting subcomplex leave PRC1.1 chromatin-bound but transcriptionally inactive, causing epigenetic reprogramming, and cooperate with Tet2 loss, Dnmt3a loss, oncogenic Kras, and Myc to produce myelodysplastic syndrome, acute leukemias, and medulloblastoma (PMID:30228234, PMID:35015684, PMID:30902969, PMID:33159179, PMID:32820036). BCOR also has developmental roles in vertebrate laterality, embryonic stem cell pluripotency, and tooth development (PMID:17517692, PMID:29337181, PMID:20563598).

Mechanistic history

Synthesis pass · year-by-year structured walk · 13 steps
  1. 2000 High

    Established BCOR as a dedicated corepressor for BCL6, answering how the BCL6 POZ domain enacts transcriptional silencing through a partner distinct from canonical SMRT/N-CoR.

    Evidence Reciprocal Co-IP, DNA-tethering repression assay, and selectivity screen against eight other POZ proteins

    PMID:10898795

    Open questions at the time
    • HDAC recruitment shown by in vivo interaction but not biochemically reconstituted
    • Genome-wide target loci not defined at this stage
  2. 2006 High

    Defined the molecular composition of the BCOR complex, revealing it recruits Polycomb proteins and two E3 ubiquitin ligases to deposit H2A monoubiquitination at BCL6 targets — the basis of PRC1.1.

    Evidence Complex purification/mass spectrometry, reciprocal Co-IP, and ChIP at endogenous BCL6 targets

    PMID:16943429

    Open questions at the time
    • Hierarchy of subunit assembly not resolved
    • Functional consequence of H2A ubiquitination on transcription not directly demonstrated
  3. 2007 Medium

    Showed BCOR has a developmental role beyond corepression by acting upstream of Pitx2c in vertebrate laterality determination.

    Evidence Side-specific morpholino knockdown in Xenopus tropicalis with in situ hybridization and phenotypic analysis

    PMID:17517692

    Open questions at the time
    • Molecular mechanism linking BCOR to Pitx2c not defined
    • Morpholino-based, single-organism evidence
  4. 2008 Medium

    Demonstrated BCOR controls developmental gene expression during stem cell differentiation and is required for normal hematopoietic and embryonic lineage contribution.

    Evidence ES cell loss-of-function alleles, in vitro differentiation, rescue by re-expression, and chimeric mouse analysis

    PMID:18795143

    Open questions at the time
    • Direct chromatin targets in ES cells not mapped
    • Parent-of-origin effect mechanism unexplained
  5. 2009 High

    Linked BCOR mutation to OFCD-associated phenotypes mechanistically, showing it maintains gene silencing via histone methylation control and represses AP-2alpha to restrain osteo-dentinogenic potential.

    Evidence Patient-derived MSCs, gain/loss-of-function, and ChIP for H3K4/H3K36 methylation at silenced targets

    PMID:19578371

    Open questions at the time
    • Relationship between histone methylation changes and PRC1.1 ubiquitination activity not connected
    • Single patient genetic background
  6. 2014 High

    Extended the BCOR repressive module to SHH signaling, showing a BCL6/BCOR/SIRT1 complex silences Gli1/Gli2 to suppress medulloblastoma.

    Evidence Reciprocal Co-IP, ChIP at Gli promoters, and an in vivo GNP-derived medulloblastoma mouse model

    PMID:25490446

    Open questions at the time
    • Interplay between SIRT1 deacetylation and PRC1.1 ubiquitination not dissected
  7. 2016 High

    Established that BCOR controls myeloid proliferation/differentiation as a PRC1.1 component by maintaining RING1B levels and H2AK119ub at HoxA loci.

    Evidence Conditional mouse knockout, expression profiling, and ChIP for H2AK119ub at HoxA, confirmed in patient AML

    PMID:26847029

    Open questions at the time
    • How BCOR loss lowers RING1B protein not mechanistically explained
  8. 2017 High

    Defined BCOR as a tumor suppressor in T-lymphocyte malignancy by restraining Myc and Notch target genes through direct promoter occupancy.

    Evidence Conditional Bcor exon 4 deletion mouse, ChIP-seq at Myc promoter, with parallel BCL6 knockout

    PMID:28262675 PMID:28827447

    Open questions at the time
    • Whether Myc repression requires PRC1.1 catalytic activity not tested directly
  9. 2018 High

    Mapped BCOR domain functions and showed C-terminal truncations sever PRC1.1 core interactions, driving Polycomb domain erosion in pluripotency and cooperating with Tet2 loss in MDS.

    Evidence Domain-deletion ChIP-seq/ATAC-seq in human ESCs and conditional Bcor exon 9-10 deletion mouse with transcriptional profiling

    PMID:29337181 PMID:30228234

    Open questions at the time
    • Precise structural basis of PRC1.1 core loss not yet resolved at this stage
  10. 2019 High

    Demonstrated BCOR loss expands myeloid progenitors and cooperates with oncogenic Kras to initiate leukemia through dysregulation of PRC1 targets including Hoxa7/9, and extended PRC1.1 function to androgen-repressed genes in prostate cancer.

    Evidence Conditional mouse knockout with transplantation and ChIP-seq; Co-IP, ChIP-seq, and knockdown in castration-resistant prostate cancer cells

    PMID:30902969 PMID:31925334

    Open questions at the time
    • Tissue-specific determinants of which PRC1.1 targets are derepressed not defined
  11. 2020 High

    Resolved the structural mechanism of PRC1.1 assembly and showed disease ITDs map to the PUFD termini to disrupt it, while defining a direct BCOR-PRC1.1/Igf2 tumor-suppressor axis in medulloblastoma.

    Evidence NMR structure with relaxation measurements and ITD-mutant binding assays; GNP mouse model with ChIP for H2AK119ub at Igf2 and rescue by ectopic Igf2

    PMID:32628469 PMID:32820036

    Open questions at the time
    • Full-length assembled PRC1.1 structure not determined
    • Generality of single-effector (Igf2) sufficiency across tumor types unknown
  12. 2020 High

    Showed BCOR's role through additional protein interactions: binding the MLL fusion partner AF9 is required for MLL-AF9 leukemogenesis, and Bcor/Dnmt3a co-loss drives erythroid leukemia via GATA factor dysregulation.

    Evidence AF9 complex structures with selective point mutants in a mouse leukemia model; conditional double-knockout mouse with transcriptomics and demethylating-agent response

    PMID:32954361 PMID:33159179

    Open questions at the time
    • Relationship between AF9-binding and PRC1.1 corepressor functions not integrated
    • Dnmt3a-cooperation experiments at Medium confidence (single lab)
  13. 2021 High

    Clarified that BCOR mutations produce a chromatin-bound but catalytically inert PRC1.1 by unlinking the RING-PCGF core from the chromatin-targeting subcomplex, defining a druggable signaling-resistance program.

    Evidence Biochemical complex assembly with BCOR mutants, ChIP-seq/RNA-seq in patient samples, and kinase-inhibitor sensitivity assays

    PMID:33468080 PMID:33862015 PMID:35015684

    Open questions at the time
    • Which targeted-kinase dependencies generalize across BCOR-mutant cancers not established
    • Point mutation K607E effects at Medium confidence without in vivo validation

Open questions

Synthesis pass · forward-looking unresolved questions
  • How BCOR-PRC1.1 target selectivity is encoded across tissues, and whether restoring PRC1.1 catalytic engagement can reverse the epigenetic reprogramming of BCOR-mutant tumors, remains unresolved.
  • No therapeutic strategy to re-couple mutant PRC1.1 enzymatic core to chromatin
  • Lineage-specific recruitment cofactors not comprehensively defined

Mechanism profile

Synthesis pass · controlled-vocabulary classification · explore literature graph →
Molecular activity
GO:0140110 transcription regulator activity 4 GO:0060090 molecular adaptor activity 3 GO:0003677 DNA binding 2 GO:0098772 molecular function regulator activity 2
Localization
GO:0000228 nuclear chromosome 4 GO:0005634 nucleus 2
Pathway
R-HSA-1266738 Developmental Biology 4 R-HSA-1643685 Disease 4 R-HSA-4839726 Chromatin organization 4 R-HSA-74160 Gene expression (Transcription) 3
Partners
AF9BCL6KDM2BNSPC1PCGF1RNF2RYBPSKP1
Complex memberships
BCL6/BCOR/SIRT1 corepressor complexPRC1.1 (non-canonical PRC1)

Evidence

Reading pass · 23 per-paper findings extracted from the source corpus
Year Finding Method Journal Conf PMIDs
2000 BCOR (BCoR) was identified as a novel corepressor that interacts selectively with the POZ domain of BCL6 but not with eight other POZ proteins tested. BCOR can function as a corepressor when tethered to DNA, potentiates BCL6 repression when overexpressed, and interacts in vivo with specific class I and II histone deacetylases (HDACs). The BCL6/BCOR interaction is mutually exclusive with BCL6 interactions with SMRT and N-CoR. Co-immunoprecipitation, DNA-tethering repression assay, overexpression, in vivo interaction studies Genes & development High 10898795
2006 BCOR forms a novel complex containing Polycomb group (PcG) proteins (RING1, RYBP, NSPC1, RNF2) and SCF ubiquitin ligase components (SKP1, FBXL10/JHDM1B). RNF2 in the complex is an E3 ligase for mono-ubiquitylation of H2A. BCOR also coimmunoprecipitates isoforms of FBXL10 containing a JmjC domain with histone H3K36 demethylase activity. BCOR complex components and mono-ubiquitylated H2A co-localize to BCL6 target genes, indicating the complex recruits PcG proteins and two distinct E3 ubiquitin ligases plus a histone demethylase to BCL6 targets. Co-immunoprecipitation, chromatin immunoprecipitation (ChIP), complex purification/mass spectrometry Molecular and cellular biology High 16943429
2009 BCOR mutation in mesenchymal stem cells (MSCs) from an OFCD patient increased osteo-dentinogenic potential. AP-2alpha was identified as a repressive target of BCOR; BCOR mutation caused abnormal activation of AP-2alpha. Gain- and loss-of-function assays demonstrated AP-2alpha mediates the increased osteo-dentinogenic capacity. BCOR maintained gene silencing through epigenetic mechanisms; its mutation increased histone H3K4 and H3K36 methylation at silenced target genes in MSCs, reactivating their transcription. Patient-derived MSC culture, gain/loss-of-function assays, ChIP for histone modifications, gene expression analysis Nature cell biology High 19578371
2007 Left-sided expression of BCOR (xtBcor) in Xenopus tropicalis is required for vertebrate laterality determination. Morpholino knockdown of xtBcor caused reversed cardiac orientation and disorganized gut patterning specifically when injected into the left side of embryos, demonstrating a left-sided requirement. xtBcor knockdown also caused ocular defects (microphthalmia, coloboma) and downregulated xtPitx2c expression, placing BCOR upstream of Pitx2c in a laterality specification pathway. Morpholino antisense knockdown in Xenopus tropicalis, in situ hybridization for Pitx2c, phenotypic analysis Human molecular genetics Medium 17517692
2008 Loss-of-function Bcor alleles in mouse embryonic stem cells result in altered expression of key developmental genes (Oct3/4, Nanog, Fgf5, Bmp4, Brachyury, Flk1) during differentiation into ectoderm, mesoderm and hematopoietic lineages, with normal expression restored upon re-expression of Bcor. Bcor loss of function shows a strong parent-of-origin effect, suggesting a requirement in extraembryonic development. Chimeric animals show reduced contribution to B and T cells and erythrocytes, and kinked/shortened tails consistent with reduced Brachyury expression. ES cell loss-of-function alleles, in vitro differentiation assays, rescue by re-expression, chimeric mouse analysis PloS one Medium 18795143
2014 BCL6, BCOR, and SIRT1 form a complex that directly represses Gli1 and Gli2 effectors of the Sonic Hedgehog (SHH) pathway. BCL6 recruits BCOR corepressor and SIRT1 deacetylase to Gli1/Gli2 promoters to suppress SHH signaling. This BCL6/BCOR/SIRT1 complex is required for neurogenesis and tumor suppression of granule neuron progenitor (GNP)-derived medulloblastoma in mice. Co-immunoprecipitation, ChIP, gain/loss-of-function in mouse GNPs and human MB cells, in vivo mouse medulloblastoma model Cancer cell High 25490446
2016 BCOR regulates myeloid cell proliferation and differentiation as a component of variant PRC1 (PRC1.1). Conditional Bcor loss-of-function in mouse bone marrow increased proliferation and differentiation of myeloid cells with upregulation of Hox genes. Bcor mutation reduced protein levels of RING1B (H2A ubiquitin E3 ligase subunit of PRC1) and reduced H2AK119 ubiquitination upstream of upregulated HoxA genes. Conditional mouse knockout, bone marrow assays, global RNA expression profiling, ChIP for H2AK119ub, protein analysis Leukemia High 26847029
2017 BCOR acts as a tumor suppressor in T lymphocyte malignancies. Mice with deletion of Bcor exon 4 (removing the BCL6-binding domain) showed augmented thymocyte proliferative capacity and strong propensity to induce acute T-cell lymphoblastic leukemia (T-ALL), mostly in a Notch-dependent manner. ChIP-seq analysis revealed BCOR is recruited to the Myc promoter and restrains its activation in thymocytes. BCOR also targeted other NOTCH1 target genes. BCL6-deficient thymocytes behaved similarly to Bcor-deficient thymocytes. Conditional Bcor exon 4 deletion mouse model, ChIP-seq, gene expression profiling, in vitro proliferation assay The Journal of experimental medicine High 28827447
2017 Bcor loss-of-function mutations are frequently found in spontaneous Eμ-Myc mouse lymphomas. Disruptive Bcor mutations cooperate with Myc overexpression (and with Cdkn2a loss, Nras, Kras) in lymphomagenesis, demonstrating a functional tumor suppressor role for Bcor in vivo in a MYC-driven malignancy. Whole-genome sequencing of spontaneous Eμ-Myc lymphomas, somatic mutation analysis Nature communications Medium 28262675
2018 BCOR is a critical component of the non-canonical PRC1.1 complex required for maintaining primed pluripotency in human embryonic stem cells (ESCs). BCOR depletion leads to erosion of Polycomb domains at key developmental loci and initiation of differentiation along endoderm and mesoderm lineages. The C-terminus of BCOR regulates assembly and targeting of the PRC1.1 complex, while the N-terminus contributes to BCOR-PRC1.1 repressor function. BCOR depletion (siRNA/shRNA) in human ESCs, ChIP-seq, ATAC-seq, RNA-seq, domain deletion mapping Cell stem cell High 29337181
2018 Bcor truncation (deletion of exons 9-10) in hematopoietic cells leads to carboxyl-terminal truncated BCOR that fails to interact with core effector components of PRC1.1. This cooperates with Tet2 deletion to cause lethal myelodysplastic syndrome (MDS) with anemia, leukocytopenia, and morphological dysplasia. Transcriptional profiling showed derepression of Cebp family myeloid regulators and HoxA cluster genes in Bcor mutant progenitors. Conditional Bcor exon 9-10 deletion mouse model, secondary transplantation, transcriptional profiling, protein interaction studies Blood High 30228234
2019 Bcor inactivation in hematopoietic stem cells (HSCs) expands myeloid progenitors and cooperates with oncogenic KrasG12D to initiate fully transplantable acute leukemia. ChIP-seq reveals differential regulation of a subset of PRC1-target genes including HSC-associated transcription factors Hoxa7/9, establishing BCOR's role in regulating cell fate decisions through PRC1 target gene control. Conditional mouse knockout, bone marrow transplantation, ChIP-seq, gene expression analysis Nature communications High 30902969
2019 BCOR interacts with androgen receptor (AR) in a hormone-dependent manner in castration-resistant prostate cancer (CRPC) cells. Genome-wide analysis shows BCOR is recruited to the majority of AR-binding chromatin sites in an androgen-dependent fashion. BCOR depletion decreases H2A K119 monoubiquitination and increases mRNA expression at androgen-repressed genes including HOX genes. BCOR depletion impairs proliferation and viability of CRPC cells, inducing apoptosis. Co-immunoprecipitation, ChIP-seq, siRNA knockdown, proliferation/apoptosis assays in CRPC cells Oncogene High 31925334
2020 BCOR directly regulates Igf2 through the PRC1.1 complex in granule neuron progenitors (GNPs). Deletion of Bcor exons 9/10 in GNPs leads to reduced H2AK119Ub at the Igf2 promoter and aberrant upregulation of Igf2. Combined with Ptch1 loss, this results in fully penetrant medulloblastomas. Ectopic Igf2 overexpression alone was sufficient to drive tumorigenesis in Ptch1+/- GNPs, placing BCOR-PRC1.1 repression of Igf2 as a key tumor suppressor mechanism. Genetically engineered mouse model (Bcor exon 9/10 deletion in GNPs), ChIP for H2AK119Ub at Igf2 promoter, ectopic Igf2 overexpression experiment, gene expression analysis Genes & development High 32820036
2020 NMR structure of the BCOR PUFD domain shows that its termini (critical for binding PCGF1 and KDM2B) are disordered. This suggests a hierarchical assembly: BCOR PUFD termini become structurally ordered upon binding PCGF1, which then allows stable association with KDM2B. Unlike BCORL1 PUFD, BCOR PUFD alone does not stably assemble with KDM2B; additional residues N-terminal to the PUFD are required. BCOR internal tandem duplications (ITDs) map to the PUFD termini and disrupt PRC1.1 assembly, suggesting loss of PRC1.1 assembly is a critical molecular event in ITD-driven tumorigenesis. NMR structure determination, 15N T2 relaxation measurements, in vitro binding studies with BCOR ITD mutant, protein biochemistry Biochemistry High 32628469
2020 BCOR mutations in myelodysplastic syndrome/AML disrupt assembly of the noncanonical PRC1.1 complex by selectively unlinking the RING-PCGF enzymatic core from the chromatin-targeting auxiliary subcomplex. BCOR-mutated PRC1.1 localizes to chromatin but lacks repressive activity, leading to epigenetic reprogramming and transcriptional activation at target loci. A set of functional PRC1.1 targets driving aberrant oncogenic signaling was defined; activation confers acquired treatment resistance while sensitizing to targeted kinase inhibition. Biochemical complex assembly studies, ChIP-seq in BCOR-mutated patient samples and cell lines, RNA-seq, kinase inhibitor sensitivity assays Blood cancer discovery High 35015684
2021 BCOR acts as a suppressor of plasmacytoid DC (pDC) and conventional DC type 2 (cDC2) numbers during Flt3 ligand-mediated emergency DC development. BCOR restricts clonal expansion, especially for cDC2s, and suppresses clonal fate potential, especially for pDCs. This was established using CRISPR-validated SIS-seq clonal multi-omics. Clonal multi-omics (SIS-seq), CRISPR validation, paired sister-clone fate assays Immunity Medium 33862015
2021 BCOR K607E mutation in T-cell lymphoma reduces binding affinity of mutant BCOR to BCL6, PCGF1, and RING1B proteins compared to wild-type BCOR. Ectopic expression of BCOR K607E significantly enhanced cell proliferation, AKT phosphorylation, and IL-2 expression with upregulated HOX and S100 protein gene expression. BCOR silencing produced similar effects, indicating the mutation acts as loss-of-function. Immunoprecipitation, western blotting, transfection/siRNA, DNA microarray, cell proliferation assay BMC cancer Medium 33468080
2020 BCOR binding to the MLL fusion partner AF9 is essential for MLL-AF9 leukemogenesis. Protein structures of AF9 complexes with BCOR and CBX8 were determined, showing binding of AF4, DOT1L, BCOR, and CBX8 to AF9 is mutually exclusive. Point mutations selectively disrupting BCOR/MLL-AF9 binding caused partial differentiation, increased proliferation, and abrogated leukemogenic potential in a mouse model. MLL-AF9 mutant deficient for BCOR binding reduces EYA1 phosphatase expression and c-Myc protein level, altering MYC-driven and SIX-regulated gene expression programs. Protein structure determination (AF9 complexes), site-directed mutagenesis, bone marrow transplantation mouse leukemia model, gene expression analysis Blood cancer discovery High 32954361
2013 BCOR (BCoR) is recruited to the CDKN1A promoter by FBI-1 (ZBTB7A) via the adaptor MBD3. FBI-1 interacts with BCOR (as well as NCoR and SMRT) to repress transcription. MBD3 differentially regulates these interactions: it decreases FBI-1/NCoR-SMRT interaction but increases FBI-1/BCoR interaction. BCoR interacts with the Mi-2/NuRD-HDAC complex, DNMTs, and HP1, contributing to epigenetic repression of CDKN1A by DNA methylation. Co-immunoprecipitation, ChIP, promoter reporter assay, siRNA knockdown Nucleic acids research Medium 23658227
2014 IRF8 interacts directly with BCOR; the α-helical region of IRF8 and the BCL6-binding domain of BCOR are required for this interaction. IRF8 also interacts directly with BCL6. IRF8 knockdown represses Bcor and enhances Bcl6 transcription in mouse B cell lymphoma cells, suggesting a regulatory relationship within a BCOR-BCL6-IRF8 complex modulating germinal center B cell transcriptional regulation. Retrovirus-based protein complementation assay screen, direct interaction validation, siRNA knockdown with qPCR The Journal of biological chemistry Medium 25331958
2010 Bcor is expressed in both dental epithelium and mesenchyme during mouse molar development. Lentivirus-mediated knockdown of Bcor in dental mesenchymal cells at E14.5 causes dentinogenesis defects and retardation of tooth root development, demonstrating a functional requirement for Bcor in the mesenchyme during early tooth development. In situ hybridization for expression profiling, lentivirus-mediated RNAi knockdown in dental mesenchyme, morphological analysis Cell and tissue research Medium 20563598
2020 Bcor knockout in mice causes impaired erythroid development (macrocytosis, anemia) and enhanced thrombopoiesis. Double knockout of Bcor and Dnmt3a leads to fully penetrant acute erythroid leukemia (AEL) characterized by expansion of c-Kit+/Ter119+ blasts. Transcriptomic analysis revealed aberrant erythroid skewing driven by epigenetic changes affecting GATA1-2 transcription factors and cell-cycle regulators (Mdm2, Tp53). Demethylating agents significantly impacted leukemic burden and survival in these mice. Conditional mouse KO, secondary transplantation, transcriptomic analysis, demethylating agent treatment in vivo Leukemia Medium 33159179

Source papers

Stage 0 corpus · 100 papers · ranked by NIH iCite citations
Year Title Journal Citations PMID
2012 A new subtype of bone sarcoma defined by BCOR-CCNB3 gene fusion. Nature genetics 354 22387997
2000 BCoR, a novel corepressor involved in BCL-6 repression. Genes & development 352 10898795
2006 Polycomb group and SCF ubiquitin ligases are found in a novel BCOR complex that is recruited to BCL6 targets. Molecular and cellular biology 284 16943429
2018 BCOR-CCNB3 Fusion Positive Sarcomas: A Clinicopathologic and Molecular Analysis of 36 Cases With Comparison to Morphologic Spectrum and Clinical Behavior of Other Round Cell Sarcomas. The American journal of surgical pathology 220 29300189
2009 BCOR regulates mesenchymal stem cell function by epigenetic mechanisms. Nature cell biology 202 19578371
2013 BCOR and BCORL1 mutations in myelodysplastic syndromes and related disorders. Blood 173 24047651
2016 Recurrent BCOR Internal Tandem Duplication and YWHAE-NUTM2B Fusions in Soft Tissue Undifferentiated Round Cell Sarcoma of Infancy: Overlapping Genetic Features With Clear Cell Sarcoma of Kidney. The American journal of surgical pathology 169 26945340
2016 BCOR Overexpression Is a Highly Sensitive Marker in Round Cell Sarcomas With BCOR Genetic Abnormalities. The American journal of surgical pathology 159 27428733
2014 BCOR-CCNB3 (Ewing-like) sarcoma: a clinicopathologic analysis of 10 cases, in comparison with conventional Ewing sarcoma. The American journal of surgical pathology 149 24805859
2019 BCOR involvement in cancer. Epigenomics 143 31150281
2013 Novel ZC3H7B-BCOR, MEAF6-PHF1, and EPC1-PHF1 fusions in ossifying fibromyxoid tumors--molecular characterization shows genetic overlap with endometrial stromal sarcoma. Genes, chromosomes & cancer 141 24285434
2016 Novel BCOR-MAML3 and ZC3H7B-BCOR Gene Fusions in Undifferentiated Small Blue Round Cell Sarcomas. The American journal of surgical pathology 132 26752546
2015 Recurrent internal tandem duplications of BCOR in clear cell sarcoma of the kidney. Nature communications 129 26573325
2017 ZC3H7B-BCOR high-grade endometrial stromal sarcomas: a report of 17 cases of a newly defined entity. Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc 127 29192652
2013 Fusion of the ZC3H7B and BCOR genes in endometrial stromal sarcomas carrying an X;22-translocation. Genes, chromosomes & cancer 115 23580382
2018 BCOR Internal Tandem Duplication in High-grade Uterine Sarcomas. The American journal of surgical pathology 111 29200103
2014 BCOR-CCNB3 fusions are frequent in undifferentiated sarcomas of male children. Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc 107 25360585
2016 Frequent BCOR aberrations in extranodal NK/T-Cell lymphoma, nasal type. Genes, chromosomes & cancer 104 26773734
2009 BCOR analysis in patients with OFCD and Lenz microphthalmia syndromes, mental retardation with ocular anomalies, and cardiac laterality defects. European journal of human genetics : EJHG 89 19367324
2014 A BCL6/BCOR/SIRT1 complex triggers neurogenesis and suppresses medulloblastoma by repressing Sonic Hedgehog signaling. Cancer cell 86 25490446
2018 A Non-canonical BCOR-PRC1.1 Complex Represses Differentiation Programs in Human ESCs. Cell stem cell 83 29337181
2017 Histological and immunohistochemical characteristics of undifferentiated small round cell sarcomas associated with CIC-DUX4 and BCOR-CCNB3 fusion genes. Virchows Archiv : an international journal of pathology 83 28197724
2017 Clinicopathologic Diversity of Undifferentiated Sarcoma With BCOR-CCNB3 Fusion: Analysis of 11 Cases With a Reappraisal of the Utility of Immunohistochemistry for BCOR and CCNB3. The American journal of surgical pathology 79 28877060
2016 BCOR regulates myeloid cell proliferation and differentiation. Leukemia 77 26847029
2022 Substantial somatic genomic variation and selection for BCOR mutations in human induced pluripotent stem cells. Nature genetics 74 35953586
2008 Role of the transcriptional corepressor Bcor in embryonic stem cell differentiation and early embryonic development. PloS one 74 18795143
2007 A novel corepressor, BCoR-L1, represses transcription through an interaction with CtBP. The Journal of biological chemistry 72 17379597
2017 Primary Renal Sarcomas With BCOR-CCNB3 Gene Fusion: A Report of 2 Cases Showing Histologic Overlap With Clear Cell Sarcoma of Kidney, Suggesting Further Link Between BCOR-related Sarcomas of the Kidney and Soft Tissues. The American journal of surgical pathology 71 28817404
2020 NTRK3 overexpression in undifferentiated sarcomas with YWHAE and BCOR genetic alterations. Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc 65 32034283
2017 Exome sequencing identifies recurrent BCOR alterations and the absence of KLF2, TNFAIP3 and MYD88 mutations in splenic diffuse red pulp small B-cell lymphoma. Haematologica 59 28751561
2019 Bcor loss perturbs myeloid differentiation and promotes leukaemogenesis. Nature communications 58 30902969
2020 Undifferentiated round cell sarcoma with BCOR internal tandem duplications (ITD) or YWHAE fusions: a clinicopathologic and molecular study. Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc 56 32372022
2016 Mutually exclusive BCOR internal tandem duplications and YWHAE-NUTM2 fusions in clear cell sarcoma of kidney: not the full story. The Journal of pathology 56 27000436
2021 BCOR gene alterations in hematologic diseases. Blood 55 33945606
2017 Recurrent BCOR internal tandem duplication and BCOR or BCL6 expression distinguish primitive myxoid mesenchymal tumor of infancy from congenital infantile fibrosarcoma. Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc 55 28256570
2017 Clear cell sarcomas of the kidney are characterised by BCOR gene abnormalities, including exon 15 internal tandem duplications and BCOR-CCNB3 gene fusion. Histopathology 53 28833375
2020 Genomic profiling of BCOR-rearranged uterine sarcomas reveals novel gene fusion partners, frequent CDK4 amplification and CDKN2A loss. Gynecologic oncology 50 32156473
2017 HGNET-BCOR Tumors of the Cerebellum: Clinicopathologic and Molecular Characterization of 3 Cases. The American journal of surgical pathology 47 28704208
2017 Internal deletion of BCOR reveals a tumor suppressor function for BCOR in T lymphocyte malignancies. The Journal of experimental medicine 47 28827447
2020 Expanding the clinicopathologic and molecular spectrum of BCOR-associated sarcomas in adults. Histopathology 46 31647130
2018 Bcor insufficiency promotes initiation and progression of myelodysplastic syndrome. Blood 45 30228234
2016 Ewing sarcoma and the new emerging Ewing-like sarcomas: (CIC and BCOR-rearranged-sarcomas). A systematic review. Histology and histopathology 45 27306060
2016 BCOR-CCNB3-positive soft tissue sarcoma with round-cell and spindle-cell histology: a series of four cases highlighting the pitfall of mimicking poorly differentiated synovial sarcoma. Histopathology 44 27228320
2007 Left-sided embryonic expression of the BCL-6 corepressor, BCOR, is required for vertebrate laterality determination. Human molecular genetics 43 17517692
2020 Endometrial stromal sarcomas with BCOR-rearrangement harbor MDM2 amplifications. The journal of pathology. Clinical research 42 32352245
2013 The proto-oncoprotein FBI-1 interacts with MBD3 to recruit the Mi-2/NuRD-HDAC complex and BCoR and to silence p21WAF/CDKN1A by DNA methylation. Nucleic acids research 42 23658227
2018 Expanding the phenotype of the X-linked BCOR microphthalmia syndromes. Human genetics 41 29974297
2015 BCOR internal tandem duplication and YWHAE-NUTM2B/E fusion are mutually exclusive events in clear cell sarcoma of the kidney. Genes, chromosomes & cancer 41 26493387
2020 High-grade transformation of low-grade endometrial stromal sarcomas lacking YWHAE and BCOR genetic abnormalities. Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc 39 32317704
2017 Genomic characterisation of Eμ-Myc mouse lymphomas identifies Bcor as a Myc co-operative tumour-suppressor gene. Nature communications 39 28262675
2022 BCOR and BCORL1 Mutations Drive Epigenetic Reprogramming and Oncogenic Signaling by Unlinking PRC1.1 from Target Genes. Blood cancer discovery 38 35015684
2019 Recurrent EP300-BCOR Fusions in Pediatric Gliomas With Distinct Clinicopathologic Features. Journal of neuropathology and experimental neurology 37 30816933
2023 A global collaboRAtive study of CIC-rearranged, BCOR::CCNB3-rearranged and other ultra-rare unclassified undifferentiated small round cell sarcomas (GRACefUl). European journal of cancer (Oxford, England : 1990) 36 36791667
2007 Characterization of Bcor expression in mouse development. Gene expression patterns : GEP 35 17344103
2016 Activation of the basal cell carcinoma pathway in a patient with CNS HGNET-BCOR diagnosis: consequences for personalized targeted therapy. Oncotarget 34 27825128
2017 BCOR-CCNB3 Undifferentiated Sarcoma-Does Immunohistochemistry Help in the Identification? Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society 31 28420319
2022 Endometrial Stromal Sarcomas With BCOR Internal Tandem Duplication and Variant BCOR/BCORL1 Rearrangements Resemble High-grade Endometrial Stromal Sarcomas With Recurrent CDK4 Pathway Alterations and MDM2 Amplifications. The American journal of surgical pathology 28 35499168
2019 BCOR Overexpression in Renal Malignant Solitary Fibrous Tumors: A Close Mimic of Clear Cell Sarcoma of Kidney. The American journal of surgical pathology 28 30864973
2017 Personalized therapy: CNS HGNET-BCOR responsiveness to arsenic trioxide combined with radiotherapy. Oncotarget 28 29371980
2020 Functional loss of a noncanonical BCOR-PRC1.1 complex accelerates SHH-driven medulloblastoma formation. Genes & development 27 32820036
2022 Intra- and extra-cranial BCOR-ITD tumours are separate entities within the BCOR-rearranged family. The journal of pathology. Clinical research 26 35174661
2021 Clonal multi-omics reveals Bcor as a negative regulator of emergency dendritic cell development. Immunity 25 33862015
2020 Superficial malignant ossifying fibromyxoid tumors harboring the rare and recently described ZC3H7B-BCOR and PHF1-TFE3 fusions. Journal of cutaneous pathology 25 32352579
2020 Imaging features and clinical course of undifferentiated round cell sarcomas with CIC-DUX4 and BCOR-CCNB3 translocations. Skeletal radiology 25 32840647
2020 BCOR Binding to MLL-AF9 Is Essential for Leukemia via Altered EYA1, SIX, and MYC Activity. Blood cancer discovery 25 32954361
2020 Fusions involving BCOR and CREBBP are rare events in infiltrating glioma. Acta neuropathologica communications 23 32493417
2019 ZC3H7B-BCOR-Rearranged Endometrial Stromal Sarcomas: A Distinct Subset Merits its Own Classification? International journal of gynecological pathology : official journal of the International Society of Gynecological Pathologists 23 29901520
2019 IGF1R Is a Potential New Therapeutic Target for HGNET-BCOR Brain Tumor Patients. International journal of molecular sciences 22 31234291
2020 OFCD syndrome and extraembryonic defects are revealed by conditional mutation of the Polycomb-group repressive complex 1.1 (PRC1.1) gene BCOR. Developmental biology 21 32692983
2020 Central nervous system high-grade neuroepithelial tumor with BCOR alteration (CNS HGNET-BCOR)-case-based reviews. Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery 20 32542405
2023 Clinical characteristics and outcomes for children, adolescents and young adults with "CIC-fused" or "BCOR-rearranged" soft tissue sarcomas: A multi-institutional European retrospective analysis. Cancer medicine 19 37212486
2020 BCOR Expression in Mullerian Adenosarcoma: A Potential Diagnostic Pitfall. The American journal of surgical pathology 19 32011345
2020 Gene of the month: BCOR. Journal of clinical pathology 19 32161069
2021 Meta-analysis of BCOR rearranged sarcomas: challenging the therapeutic approach. Acta oncologica (Stockholm, Sweden) 18 33630701
2018 Preoperative diagnosis of clear cell sarcoma of the kidney by detection of BCOR internal tandem duplication in circulating tumor DNA. Genes, chromosomes & cancer 17 30126017
2021 Loss-of-function mutations in BCOR contribute to chemotherapy resistance in acute myeloid leukemia. Experimental hematology 16 34333045
2020 Structure and Role of BCOR PUFD in Noncanonical PRC1 Assembly and Disease. Biochemistry 16 32628469
2020 Bcor deficiency perturbs erythro-megakaryopoiesis and cooperates with Dnmt3a loss in acute erythroid leukemia onset in mice. Leukemia 16 33159179
2019 Engineered Bcor mutations lead to acute leukemia of progenitor B-1 lymphocyte origin in a sensitized background. Blood 15 30992267
2023 Pediatric BCOR-Altered Tumors From Soft Tissue/Kidney Display Specific DNA Methylation Profiles. Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc 14 36853789
2022 BCOR variants are associated with X-linked recessive partial epilepsy. Epilepsy research 14 36279688
2020 BCOR-coupled H2A monoubiquitination represses a subset of androgen receptor target genes regulating prostate cancer proliferation. Oncogene 14 31925334
2014 Oculofaciocardiodental syndrome: novel BCOR mutations and expression in dental cells. Journal of human genetics 14 24694763
2010 Function analysis of mesenchymal Bcor in tooth development by using RNA interference. Cell and tissue research 14 20563598
2023 Spectrum of Histopathological, Immunohistochemical, Molecular and Radiological Features in 12 Cases of BCOR::CCNB3-positive Sarcomas With Literature Review. International journal of surgical pathology 13 36591870
2022 Soft Tissue and Visceral Organ Sarcomas With BCOR Alterations. Journal of pediatric hematology/oncology 13 35537005
2022 Mutations in KMT2C, BCOR and KDM5C Predict Response to Immune Checkpoint Blockade Therapy in Non-Small Cell Lung Cancer. Cancers 13 35681795
2021 Imaging of bone and soft tissue BCOR-rearranged sarcoma. Skeletal radiology 13 33388948
2021 The mutation of BCOR is highly recurrent and oncogenic in mature T-cell lymphoma. BMC cancer 13 33468080
2023 CNS tumor with EP300::BCOR fusion: discussing its prevalence in adult population. Acta neuropathologica communications 12 36782314
2021 Detection of BCOR gene rearrangement in Ewing-like sarcoma: an important diagnostic tool. Diagnostic pathology 12 34103053
2019 BCOR-CCNB3 fusion-positive clear cell sarcoma of the kidney. Pediatric blood & cancer 12 31876361
2014 Interferon regulatory factor 8 (IRF8) interacts with the B cell lymphoma 6 (BCL6) corepressor BCOR. The Journal of biological chemistry 12 25331958
2021 Assessment of BCOR Internal Tandem Duplications in Pediatric Cancers by Targeted RNA Sequencing. The Journal of molecular diagnostics : JMD 11 34325058
2020 Fine-Needle Aspiration Features of BCOR-CCNB3 Sarcoma. American journal of clinical pathology 11 31679010
2013 Overexpression of the transcriptional repressor complex BCL-6/BCoR leads to nuclear aggregates distinct from classical aggresomes. PloS one 11 24146931
2021 Specific and Sensitive Diagnosis of BCOR-ITD in Various Cancers by Digital PCR. Frontiers in oncology 10 33718245
2024 Genetic landscape and clinical outcomes of patients with BCOR mutated myeloid neoplasms. Haematologica 9 38299584
2023 KDM2B-Rearranged Soft Tissue Sarcomas Expand the Concept of BCOR-Associated Sarcoma. Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc 9 37634866
2020 Primary bone sarcoma with BCOR internal tandem duplication. Virchows Archiv : an international journal of pathology 9 31900635

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