Affinage

STAG2

Cohesin subunit SA-2 · UniProt Q8N3U4

Length
1231 aa
Mass
141.3 kDa
Annotated
2026-06-10
100 papers in source corpus 34 papers cited in narrative 34 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

STAG2 (SA2) is a core subunit of one of two mutually exclusive variant cohesin complexes, where it confers functions distinct and non-redundant from its paralog STAG1: cohesin-SA2 specifically maintains centromeric sister chromatid cohesion whereas cohesin-SA1 maintains telomere cohesion (PMID:19822671). During mitosis, Polo-like kinase 1 phosphorylates SA2 at multiple sites, an event required for cohesin dissociation from chromosome arms in prophase/prometaphase but dispensable for separase-mediated cleavage (PMID:15737063). STAG2 is a sequence-independent DNA-binding protein that engages dsDNA, ssDNA, and replication/repair intermediates, as well as RNA, RNA:DNA hybrids, and R-loops, and supports DNA replication fork progression, the cohesin–replisome interaction, SMC3 acetylation, and homologous-recombination repair via its diverged C-terminal region (PMID:24324008, PMID:29175904, PMID:30975996, PMID:32352519). As the direct interface between cohesin and the CTCF C-terminus, STAG2 mediates chromatin insulation, and beyond CTCF sites it occupies enhancers and Polycomb-marked regions to promote cell-type-specific enhancer–promoter contacts and Polycomb domain organization that STAG1 cannot substitute for (PMID:21444719, PMID:29867216, PMID:31216471). Through these genome-folding activities STAG2 controls lineage-specification and differentiation programs in hematopoietic, oligodendrocyte, and other contexts (PMID:31495782, PMID:35959892, PMID:39084219). STAG2 is a recurrently inactivated tumor suppressor whose loss drives aneuploidy in some settings and aneuploidy-independent oncogenic 3D-genome rewiring in others, reprogramming enhancer–promoter contacts (including EWS::FLI1 neo-enhancers in Ewing sarcoma), Polycomb domains, and signaling outputs to alter migration, drug response, and differentiation (PMID:21852505, PMID:34129824, PMID:24121791, PMID:39487368, PMID:38705393). STAG2-mutant cells are dependent on STAG1, generating a robust synthetic-lethal interaction, and STAG2 loss confers sensitivity to PARP and ATR inhibitors via its replication/HR roles (PMID:30975996, PMID:28691904). A familial germline STAG2 missense mutation (p.Ser327Asn) that disrupts binding to SCC1 and cohesin regulators causes a cohesinopathy (PMID:29263825).

Mechanistic history

Synthesis pass · year-by-year structured walk · 16 steps
  1. 2002 Medium

    Established that STAG2 is present in meiotic cells, indicating the somatic cohesin SA-subunit operates alongside the meiosis-specific STAG3 complex rather than STAG3 acting alone.

    Evidence Immunofluorescence localization of STAG2 and Rad21 during mouse spermatogenesis and oogenesis

    PMID:12034751

    Open questions at the time
    • Localization-only; no functional test of STAG2's meiotic requirement
    • No reconstitution of a STAG2-containing meiotic complex
  2. 2005 High

    Resolved how cohesin leaves chromosome arms in early mitosis, showing PLK1 phosphorylation of SA2 is the trigger for the prophase pathway, separate from separase cleavage.

    Evidence Mass spectrometry mapping of mitotic phosphosites plus non-phosphorylatable SA2 mutants expressed at physiological levels in human cells

    PMID:15737063

    Open questions at the time
    • Does not define how phosphorylation alters cohesin–DNA engagement structurally
    • Relationship to WAPL-dependent release not addressed
  3. 2009 High

    Defined SA1 vs SA2 division of labor, showing they hold distinct chromosomal positions (SA2 centromere, SA1 telomere) and are non-redundant.

    Evidence Reciprocal RNAi depletion of SA1 or SA2 with imaging of centromere vs telomere cohesion in human cells

    PMID:19822671

    Open questions at the time
    • Molecular basis for position-specific targeting unknown
    • Whether other cohesin regulators dictate the split not addressed
  4. 2011 High

    Identified STAG2 as the direct molecular cause of cancer aneuploidy and as the physical CTCF–cohesin interface, linking a single subunit to both chromosome segregation and genome insulation.

    Evidence AAV-mediated inactivation and correction of endogenous STAG2 in human/glioblastoma lines; Co-IP and insulator/imprinting assays mapping CTCF C-terminal SA2-binding sites

    PMID:21444719 PMID:21852505

    Open questions at the time
    • Aneuploidy causation later shown context-dependent (not universal)
    • Structure of the CTCF–SA2 contact not resolved
  5. 2013 High

    Distinguished STAG2's tumor-suppressor mechanism from chromosome counting and assigned cohesin-SA2 a specialized role at DNA damage sites.

    Evidence STAG2 knockdown/re-expression colony assays in bladder cancer; laser micro-irradiation, depletion, domain-swap chimeras and HR assays for SA2 vs SA1

    PMID:24121791 PMID:24324008

    Open questions at the time
    • Aneuploidy-independent tumor-suppressive mechanism not yet defined in 2013
    • Recruitment partners at damage sites incompletely mapped
  6. 2014 Medium

    Connected STAG2 loss to mitotic error through kinetochore–microtubule mis-regulation rather than simple cohesion loss, and showed errors are correctable by destabilizing kMT attachments.

    Evidence STAG2 depletion with live imaging, kinetochore protein immunofluorescence, and MCAK/Kif2B overexpression rescue in human cells

    PMID:25074805

    Open questions at the time
    • Single-lab phenotype
    • Mechanism linking SA2 to Bub1/CPC localization unresolved
  7. 2015 Medium

    Mapped Sororin to SA2, defining SA2 as the cohesin anchor through which sister chromatid cohesion is protected.

    Evidence Co-IP of Sororin C-terminal truncations with SA2 and chromosome separation assays

    PMID:25608232

    Open questions at the time
    • Two-method single-lab study
    • Structural interface not determined
  8. 2016 High

    Distinguished functional consequences of STAG2 mutation classes and linked STAG2 loss to therapy resistance via CTCF-controlled DUSP6/MAPK signaling.

    Evidence Knock-in of nine tumor-derived mutations with Co-IP of WAPL/PDS5; shRNA knockdown with CTCF ChIP, DUSP6/ERK analysis and BRAFi xenografts in melanoma

    PMID:26871722 PMID:27500726

    Open questions at the time
    • How missense mutants alter regulator binding without disrupting cohesion mechanistically unclear
    • DUSP6/MAPK axis tested in melanoma only
  9. 2017 High

    Established STAG2–STAG1 synthetic lethality as a therapeutic vulnerability, defined STAG2 as a sequence-independent DNA-binding protein recognizing repair intermediates, and revealed alternative telomere maintenance upon loss.

    Evidence Isogenic STAG1 inactivation with cohesion/viability rescue; single-molecule AFM/tightrope and anisotropy DNA-binding assays; telomere FISH/SCE and senescence assays

    PMID:28691904 PMID:28819029 PMID:29175904

    Open questions at the time
    • In vivo DNA-binding contribution to genome folding not directly tested
    • Mechanism of telomere recombination induction unresolved
  10. 2018 High

    Defined SA2-specific genome-organizing activity at enhancers beyond CTCF and connected STAG2 loss to innate-immune activation through cytosolic DNA sensing.

    Evidence ChIP-seq/Hi-C/RNA-seq distinguishing SA2 enhancer contacts from SA1 TAD boundaries; CRISPR KO with cGAS-STING/JAK-STAT/ISG readouts in cells and enteroids

    PMID:29662124 PMID:29867216

    Open questions at the time
    • Whether DNA-binding activity directly underlies enhancer occupancy untested
    • Source of cytosolic DNA upon STAG2 loss not fully defined
  11. 2019 High

    Mechanistically tied STAG2 to replication fork progression and SMC3 acetylation, to Polycomb domain compaction, and to lineage-gene accessibility in hematopoiesis.

    Evidence DNA fiber assays, cohesin–replisome Co-IP and PARP/ATR sensitivity; ChIP-seq/Hi-C of SA2 and PRC1/2 in mESCs; conditional KO with ChIP/ATAC/RNA-seq in murine HSPCs

    PMID:30975996 PMID:31216471 PMID:31495782

    Open questions at the time
    • How SA2 enables SMC3 acetylation molecularly unresolved
    • PRC1 recruitment mechanism by SA2 not defined
  12. 2020 High

    Extended STAG2's nucleic-acid binding to RNA and R-loops, and demonstrated STAG2-dependent promoter loops drive a tissue program (myelination) in vivo, while chronic loss reshapes loop-domain architecture in AML.

    Evidence AFM/tightrope/anisotropy RNA-binding with ChIP-DRIP overlap; conditional Stag2 KO mice with oligodendrocyte RNA-seq/chromatin-loop mapping; HiChIP/RNA-seq with MEKi sensitivity in AML model

    PMID:32352519 PMID:32883299 PMID:35959892

    Open questions at the time
    • Functional role of RNA/R-loop binding in vivo unresolved
    • Single AML model for compartment changes
  13. 2021 High

    Resolved STAG2-specific control of EWS::FLI1 neo-enhancer communication, bladder lineage programs, and loop-extrusion dynamics, showing STAG1 cannot compensate at enhancer-rich regions.

    Evidence ChIP-seq/Hi-C/RNA-seq in isogenic Ewing sarcoma and bladder cancer cells with migration/metastasis assays

    PMID:33930311 PMID:34129824 PMID:34648034

    Open questions at the time
    • Why STAG1-cohesin avoids enhancer-rich sites unresolved
    • Direct versus indirect effects on EWS::FLI1 activity debated within the data
  14. 2022 High

    Linked STAG2-loss-driven 3D-genome rewiring to an IRF9/type-I-interferon/PD-L1 axis, connecting genome organization to immune evasion.

    Evidence STAG2 ChIP-seq, STAG1 ChIP-seq, H3K27ac HiChIP and RNA-seq in CRISPR-KO melanoma cells with PD-L1 analysis

    PMID:35388001

    Open questions at the time
    • Causal contribution of IRF9 to immune evasion in vivo not established
    • Generality beyond melanoma untested
  15. 2023 Medium

    Identified the PAXIP1–STAG2 co-regulatory axis for hormone-receptor transcription, a KMT5A/H4K20me–BRCA1-BARD1 mechanism for the HR defect, and a c-Myc/glutamine-metabolism vulnerability.

    Evidence CRISPR screen with GR ChIP/Hi-C/RNA-seq and epistasis; CRISPR KO with KMT5A/H4K20me and BRCA1-BARD1 recruitment plus PARP/ATM sensitivity; STAG2 knockdown with c-Myc/ERK-AKT-GSK3β and BPTES assays

    PMID:37070193 PMID:37479689 PMID:37985839

    Open questions at the time
    • How STAG2 loss restores KMT5A expression mechanistically unclear
    • Metabolic axis tested in thyroid cancer only
  16. 2024 High

    Solidified STAG2 as the uniquely tumor-suppressive cohesin subunit acting through a STAG2-PAXIP1/PAGR1 axis, and high-resolution looping studies showed STAG2 correction reverses Polycomb activation and STAG1-cohesin makes larger, more processive loops.

    Evidence In vivo CRISPR barcoding epistasis in KRAS lung cancer with multi-omics; Capture Hi-C and multi-subunit ChIP-seq in Ewing sarcoma; endogenous STAG2 correction with Hi-C/H3K27me3 ChIP in GBM; cohesin ChIP/Hi-C in AML patients and HSPC depletion

    PMID:38705393 PMID:39084219 PMID:39487368 PMID:39652422

    Open questions at the time
    • Molecular basis of STAG1 vs STAG2 loop-processivity difference unresolved
    • How PAXIP1/PAGR1 stabilize STAG2-cohesin on chromatin not fully defined

Open questions

Synthesis pass · forward-looking unresolved questions
  • It remains unresolved how STAG2's intrinsic DNA/RNA-binding activity, its CTCF/enhancer targeting, and its loop-extrusion behavior are mechanistically coupled, and what determines the context-specific switch between aneuploidy-driven and aneuploidy-independent oncogenic outcomes.
  • No structural model of the cohesin-SA2–CTCF or SA2–DNA complex in the corpus
  • Determinants of STAG1 vs STAG2 site selectivity unknown
  • Unifying rule for tissue-specific phenotypic outcomes of STAG2 loss lacking

Mechanism profile

Synthesis pass · controlled-vocabulary classification · explore literature graph →
Molecular activity
GO:0140110 transcription regulator activity 3 GO:0003677 DNA binding 2 GO:0005198 structural molecule activity 2 GO:0003723 RNA binding 1 GO:0060090 molecular adaptor activity 1
Localization
GO:0005694 chromosome 3 GO:0000228 nuclear chromosome 2 GO:0005634 nucleus 2
Pathway
R-HSA-1643685 Disease 5 R-HSA-4839726 Chromatin organization 5 R-HSA-1640170 Cell Cycle 4 R-HSA-74160 Gene expression (Transcription) 4 R-HSA-73894 DNA Repair 3 R-HSA-168256 Immune System 2 R-HSA-69306 DNA Replication 1
Complex memberships
cohesin (cohesin-SA2)

Evidence

Reading pass · 34 per-paper findings extracted from the source corpus
Year Finding Method Journal Conf PMIDs
2005 Phosphorylation of SA2 (STAG2) by Polo-like kinase 1 (Plk1) is essential for dissociation of cohesin from chromosome arms during prophase and prometaphase. Non-phosphorylatable SA2 mutants expressed at physiological levels in human cells phenocopied Plk1 depletion, preventing cohesin arm dissociation in early mitosis, while SA2 phosphorylation was not required for cohesin cleavage by separase. Multiple mitosis-specific phosphorylation sites on SA2 were identified by mass spectrometry. Mass spectrometry identification of phosphorylation sites; stable expression of non-phosphorylatable SA2 mutants in human cells; biochemical in vitro experiments; phenotypic analysis of mitosis PLoS biology High 15737063
2011 Mutational inactivation of STAG2 in a near-diploid human cell line causes chromatid cohesion defects and aneuploidy. Conversely, targeted correction of endogenous mutant STAG2 alleles in aneuploid glioblastoma cell lines enhanced chromosomal stability, establishing STAG2 loss as a direct cause of aneuploidy in cancer. Targeted gene inactivation (AAV-mediated) in near-diploid human cells; targeted correction of endogenous STAG2 mutations in glioblastoma cell lines; chromosome counting and cohesion assays Science (New York, N.Y.) High 21852505
2009 Cohesin-SA2 and cohesin-SA1 have distinct and non-redundant roles in cohesion: SA2-depleted cells lose centromere cohesion prematurely while telomere cohesion remains normal, whereas SA1-depleted cells specifically lose sister telomere cohesion. RNAi depletion of SA1 or SA2 in human cells; fluorescence imaging of telomere and centromere cohesion; phenotypic analysis of chromosome morphology The Journal of cell biology High 19822671
2011 CTCF interacts directly with the cohesin subunit SA2 via specific sites in the C-terminal tail of CTCF; all other cohesin components are recruited through their interaction with SA2. CTCF mutants lacking these SA2-binding sites lose insulation activity and disrupt imprinted gene expression, demonstrating that SA2 is the direct CTCF-cohesin interface. Co-immunoprecipitation; in vivo expression of CTCF C-terminal deletion and point mutants; reporter assays for insulator function; analysis of imprinted gene expression (IGF2-H19) Molecular and cellular biology High 21444719
2013 Cohesin-SA2 (not SA1) is the primary complex co-recruited with the cohesin-loading factor NIPBL to DNA damage sites in an S/G2-phase-specific manner; the diverged C-terminal region of SA2 confers this activity. Depletion of SA2 but not SA1 decreased sister chromatid homologous recombination repair and affected repair pathway choice, establishing cohesin-SA2 as the functionally specialized complex for DNA damage site recruitment and HR repair. Laser micro-irradiation and live-cell imaging; siRNA depletion of SA1 vs. SA2; domain-swap chimera experiments (SA1 C-terminus replaced with SA2 C-terminus); sister chromatid HR repair assays; intra-S checkpoint assays Molecular and cellular biology High 24324008
2017 SA2 (STAG2) is a sequence-independent DNA-binding protein that binds both dsDNA and ssDNA (with higher affinity for ssDNA), can switch between 1D diffusion mode on dsDNA and stable binding at ssDNA gaps, and specifically recognizes DNA replication and DSB repair intermediates (ssDNA overhangs, flaps, forks, gaps, and double-stranded ends) but not centromeric or telomeric sequences. SA2 loss leads to a defect in homologous recombination-mediated DSB repair. Single-molecule atomic force microscopy; single-molecule fluorescence microscopy (DNA tightrope assay); fluorescence anisotropy binding measurements; HR repair assays after SA2 loss The Journal of biological chemistry High 29175904
2018 Cohesin-SA2 localizes to CTCF sites and additionally to enhancers lacking CTCF, where it promotes cell-type-specific enhancer–promoter contacts. Cohesin-SA1 preferentially stabilizes TAD boundaries together with CTCF. Loss of SA2 rewires local chromatin contacts and alters gene expression at tissue-specific loci that cannot be rescued by SA1. ChIP-seq for SA1, SA2, CTCF; Hi-C genome-wide chromosome conformation capture; RNA-seq after SA2 depletion; comparison of isogenic SA2-depleted vs. wild-type human cells Nature structural & molecular biology High 29867216
2019 STAG2 is essential for DNA replication fork progression; STAG2 inactivation causes replication fork stalling and collapse, disrupts the interaction between the cohesin ring and the replication machinery, and prevents establishment of SMC3 acetylation. As a consequence, STAG2 loss confers synthetic lethality with DSB repair genes and sensitivity to PARP and ATR inhibitors. DNA fiber assays for replication fork progression; co-immunoprecipitation of cohesin with replication machinery; SMC3 acetylation assays; CRISPR-based isogenic cell lines; drug sensitivity assays (PARP, ATR inhibitors) Nature communications High 30975996
2019 Stag2 deletion in hematopoietic stem and progenitor cells (HSPCs) reduces chromatin accessibility and transcription of lineage-specification genes (including Ebf1 and Pax5), leading to increased self-renewal and impaired differentiation (particularly B cell lineage commitment). ChIP-seq shows that Stag2 and Stag1 share most binding sites, but a subset of Stag2 sites are unoccupied by Stag1 even in Stag2-deficient cells. Conditional Stag2 knockout in murine HSPCs; ChIP-seq for Stag1 and Stag2; ATAC-seq for chromatin accessibility; RNA-seq; hematopoietic differentiation assays Cell stem cell High 31495782
2019 Cohesin-SA2 facilitates Polycomb domain compaction through PRC1 recruitment and promotes long-range interaction networks between distant Polycomb-bound promoters in mouse embryonic stem cells. Cohesin-SA1 disrupts these networks while preserving TAD borders. ChIP-seq for SA1, SA2, PRC1/2 components; Hi-C; RNA-seq after selective depletion of SA1 or SA2 in mESCs Cell reports High 31216471
2018 STAG2 deficiency causes spontaneous genomic DNA damage that activates robust interferon expression via the cGAS-STING cytosolic DNA-sensing pathway, leading to JAK-STAT signaling and broad ISG expression that confers resistance to viral infection including rotavirus. Genome-wide CRISPR-Cas9 screen for rotavirus host factors; STAG2 knockout in cell lines and human intestinal enteroids; measurement of DNA damage, IFN expression, cGAS-STING pathway activation; JAK-STAT and ISG analysis Nature communications High 29662124
2017 STAG1 displays a strong synthetic lethal interaction with STAG2 in cancer cells. Mechanistically, STAG1 loss abrogates sister chromatid cohesion specifically in STAG2-mutated cells (not wild-type), leading to mitotic catastrophe and apoptosis. Restoration of STAG2 expression in STAG2-mutant cells alleviates the dependency on STAG1. CRISPR/shRNA-based STAG1 inactivation in isogenic STAG2-mutant vs. wild-type bladder cancer and Ewing sarcoma cell lines; sister chromatid cohesion assays; cell viability and mitosis assays; STAG2 re-expression rescue experiments eLife High 28691904
2014 STAG2 depletion does not impair bipolar spindle formation but causes excessive centromere stretch and hyperstabilization of kinetochore-microtubule (kMT) attachments, along with mislocalization of Bub1 kinase, Bub3, and the chromosome passenger complex. Strategic destabilization of kMT attachments in STAG2-mutant tumor cells by overexpression of MCAK/KIF2C and Kif2B decreased lagging chromosomes and chromosome missegregation. STAG2 depletion in human cells; live-cell imaging of mitosis; immunofluorescence for kinetochore proteins and CPC; kMT attachment destabilization by MCAK/Kif2B overexpression; chromosome segregation assays Journal of cell science Medium 25074805
2015 The C-terminus of Sororin (last 12 amino acid residues) is required for Sororin to bind the cohesin subunit SA2. Deletion of the last 12 residues inhibits Sororin–SA2 interaction and causes precocious chromosome separation, identifying SA2 as the cohesin anchor for Sororin in sister chromatid cohesion protection. Co-immunoprecipitation of Sororin deletion mutants with SA2; chromosome separation assays in cells expressing C-terminal truncations of Sororin Cell cycle (Georgetown, Tex.) Medium 25608232
2002 STAG2 (and Rad21) are present in meiotic cells and associate with chromosomes during diplotene stage of meiosis, suggesting that a cohesin complex containing Rad21 and STAG2 cooperates with the STAG3-specific meiotic complex to maintain sister chromatid cohesion during meiosis—not solely the STAG3 complex. Immunofluorescence localization of STAG2 and Rad21 during mouse spermatogenesis and oogenesis; co-localization analysis with STAG3 EMBO reports Medium 12034751
2017 A familial germline STAG2 missense mutation (p.Ser327Asn) disrupts STAG2 binding to SCC1 and other cohesin subunits/regulators when expressed in human cells in vivo, causing a cohesinopathy. S327 is located at a conserved site crucial for binding SCC1 and cohesin regulators. Paradoxically, recombinant STAG2 p.Ser327Asn binds normally to SCC1, WAPL, and SGO1 in vitro, suggesting unknown in vivo mechanisms regulate the STAG2–SCC1 interaction. Co-immunoprecipitation of mutant vs. wild-type STAG2 with cohesin subunits in human cells; in vitro binding assays with recombinant proteins; cell cycle analysis; gene expression profiling of patient-derived cells NPJ genomic medicine Medium 29263825
2020 SA1 (STAG1) and SA2 (STAG2) directly bind to RNA (ssRNA, dsRNA, RNA:DNA hybrids, and R-loops) as well as to dsDNA regions containing RNA. SA1 and SA2 binding sites from ChIP-seq significantly overlap with R-loop sites from DRIP-seq, and the majority of R-loop-localized SA1/SA2 are also sites where other cohesin complex subunits bind. Atomic force microscopy (AFM); single-molecule DNA tightrope assay; fluorescence anisotropy; bulk biochemical RNA/DNA binding assays; overlap analysis of ChIP-seq and DRIP-seq data Nucleic acids research High 32352519
2021 STAG2-containing cohesin complexes occupy enhancer and PRC2-marked regulatory regions. STAG2 loss leads to compensatory increase in cohesin-STAG1 complexes, but not at enhancer-rich regions, resulting in reprogramming of cis-chromatin interactions. This alters the oncogenic program driven by EWS/FLI1 (via altered enhancer-promoter contacts) and disrupts PRC2-mediated gene repression, converging to enhance metastatic potential of Ewing sarcoma. ChIP-seq for STAG1, STAG2, H3K27ac; Hi-C; RNA-seq in STAG2 knockout Ewing sarcoma cells; xenograft metastasis assays Cancer cell High 34129824
2021 STAG2 loss-of-function strongly alters CTCF-anchored dynamic loop extrusion and dramatically decreases promoter-enhancer interactions—particularly at genes regulated by EWSR1-FLI1 at GGAA microsatellite neo-enhancers—without significantly changing EWSR1-FLI1, CTCF/cohesin, or H3K27ac binding patterns. Down-modulation of cis-mediated EWSR1-FLI1 activity by STAG2-LOF is associated with enhanced migration and invasion. ChIP-seq; Hi-C; RNA-seq in isogenic Ewing sarcoma cells with/without STAG2 LOF; migration/invasion assays Cancer cell High 33930311
2022 STAG2 depletion in melanoma cells leads to TAD expansion and enhanced H3K27ac-associated DNA loop formation at sites where STAG2 binding switches to STAG1. IRF9 is identified as a direct target of STAG2 regulation; STAG2 loss activates IRF9 via altered 3D genome organization, which in turn enhances type I interferon signaling and increases PD-L1 expression, potentially promoting immune evasion. RNA-seq; STAG2 ChIP-seq; H3K27ac HiChIP; STAG1 ChIP-seq; STAG2 CRISPR KO in melanoma cells; PD-L1 expression analysis Nature communications High 35388001
2023 PAXIP1 is required for stability of cohesin (including STAG2) on chromatin and for its localization to glucocorticoid receptor (GR)-occupied sites. PAXIP1 and STAG2 converge to maintain 3D genome architecture (enhancer-promoter interactions) required for GR-driven transcription; PAXIP1/STAG2 depletion alters the GR transcriptome without changing the GR cistrome, establishing a PAXIP1-STAG2 co-regulator axis for stress hormone signaling. FACS-based genome-wide CRISPR screen; ChIP-seq for GR, STAG2, cohesin; Hi-C; RNA-seq; STAG2 and PAXIP1 co-depletion in lung cancer cells; epistasis experiments Nucleic acids research High 37070193
2023 STAG2 knockout increases DSBs and chromosomal aberrations by reducing homologous recombination repair. Mechanistically, STAG2 deficiency restores expression of KMT5A, which methylates H4K20 (H4K20me0 to H4K20me1), thereby decreasing recruitment of BRCA1-BARD1 to chromatin and impairing HR. STAG2 loss confers hypersensitivity to ATM inhibitor, PARP inhibitor, and their combination. STAG2 CRISPR KO; HR repair assays; γH2AX and chromosomal aberration quantification; KMT5A expression and H4K20 methylation analysis; BRCA1-BARD1 chromatin recruitment assays; drug sensitivity assays Advanced science (Weinheim, Baden-Wurttemberg, Germany) Medium 37985839
2020 In mice, Stag2 ablation in the nervous system (conditional KO in oligodendrocytes) impairs myelination: Stag2-cKO oligodendrocytes show delayed maturation and downregulation of myelination-related genes. Mechanistically, STAG2-cohesin generates promoter-anchored chromatin loops at myelination-promoting genes to facilitate their transcription; Stag2 loss reduces these promoter-anchored loops at downregulated myelination genes. Conditional Stag2 KO mice; RNA-seq of oligodendrocytes; ChIP-seq/HiChIP for cohesin and chromatin loops; histological analysis of myelination; behavioral and survival assessment eLife High 35959892
2024 STAG2 is uniquely tumor-suppressive among all core and auxiliary cohesin components in oncogenic KRAS-driven lung tumorigenesis in vivo. PAXIP1 and PAGR1 are epistatic to STAG2 in this context: their tumor-suppressive effects are highly correlated with STAG2, and STAG2 inactivation elicits gene expression, chromatin accessibility, and 3D genome conformation changes shared with PAXIP1-deficient cells, establishing a STAG2-PAXIP1/PAGR1 tumor-suppressive axis. Somatic CRISPR-Cas9 genome editing with tumor barcoding in autochthonous KRAS-driven lung cancer mouse model; epistasis in vivo; RNA-seq; ATAC-seq; Hi-C in lung cancer cell lines The Journal of experimental medicine High 39652422
2016 Loss of STAG2 in melanoma cells inhibits CTCF-mediated expression of DUSP6, leading to reactivation of MAPK signaling (ERK1/2), which confers resistance to BRAF inhibitors. STAG2 knockdown decreased sensitivity of BRAF(V600E)-mutant melanoma cells and xenograft tumors to BRAFi. shRNA knockdown of STAG2 or STAG3 in BRAF(V600E) melanoma cell lines; xenograft models; CTCF ChIP; DUSP6 expression analysis; ERK phosphorylation assays; sequencing of BRAFi-resistant patient tumors Nature medicine Medium 27500726
2016 Many tumor-derived STAG2 missense mutations retain the ability to interact with the cohesin ring, but the presence of mutant STAG2 reduces the ability of regulatory subunits WAPL, PDS5A, and PDS5B to interact with the core cohesin ring. Nonsense STAG2 mutations cause cohesion defects and some anaphase defects, while missense mutations do not impair cohesion or chromosome segregation, suggesting distinct functional consequences of different mutation classes. AAV-mediated introduction of nine tumor-derived STAG2 mutations into the endogenous locus; co-immunoprecipitation of WAPL, PDS5A, PDS5B; sister chromatid cohesion assays; anaphase analysis; chromosome counting PLoS genetics High 26871722
2013 STAG2 loss in bladder cancer cells (knockdown) does not increase aneuploidy, while STAG2 reintroduction into non-expressing bladder cancer cells led to reduced colony formation, indicating a tumor suppressor role through mechanisms independent of aneuploidy prevention. siRNA knockdown of STAG2 in bladder cancer cells with karyotype analysis; stable re-expression of STAG2 in STAG2-negative cells; colony formation assays Nature genetics Medium 24121791
2020 Chronic loss of STAG2 in AML cells leads to loss of smaller chromatin loop domains and formation/maintenance of large domains, altering genome compartmentalization. These structural changes result in deregulated gene expression including the HOXA locus and MAPK signaling pathway, and increase sensitivity to MEK inhibition. RNA-seq; ChIP-seq; HiChIP in a chronic STAG2 loss AML cell model; MEK inhibitor sensitivity assays Journal of translational medicine Medium 32883299
2021 STAG2 loss in bladder cancer cells reduces short- and mid-range chromatin interactions engaging genes (more so than STAG1-mediated contacts), results in down-regulation of the luminal urothelial gene signature and up-regulation of the basal transcriptional program. Contacts lost upon STAG2 depletion preferentially occur within silent chromatin domains and are associated with de-repression of lineage-specifying genes, suggesting STAG2-mediated looping maintains the basal program in a silent state. ChIP-seq for STAG1, STAG2; Hi-C; RNA-seq; STAG2 knockdown in RT112 bladder cancer cells; integration of genomic and gene expression data Nucleic acids research Medium 34648034
2024 STAG2 mutations in AML alter cohesin occupancy at specific loci, reduce gene expression and local chromatin activation at affected sites, and disrupt spatial chromatin looping. These effects are not compensated by STAG1-cohesin. STAG2 depletion (not STAG1 depletion) in primary human HSPCs impairs differentiation and maintains HSPC-like gene expression, mimicking STAG2-mutant AML. Cohesin ChIP-seq, RNA-seq, and Hi-C in AML patient samples; STAG2 and STAG1 depletion in primary human HSPCs; differentiation assays Cell reports High 39084219
2024 In Ewing sarcoma, cohesin-STAG2 facilitates communication between EWS::FLI1-bound GGAA microsatellite neo-enhancers and their target promoters. STAG2 loss severely decreases total chromatin-bound cohesin (NIPBL levels unchanged), alters CTCF-dependent chromatin contacts at STAG2-dependent signature genes unrelated to EWS::FLI1, and STAG1 cannot compensate. A STAG2-dependent gene signature is associated with worse prognosis. Capture Hi-C; ChIP-seq for STAG1, STAG2, CTCF, cohesin subunits; RNA-seq in isogenic STAG2-KO Ewing sarcoma cells; patient transcriptomic data analysis EMBO reports High 39487368
2024 STAG2 mutation correction in GBM cell lines alters expression of ~10% of genes, predominantly negatively regulated by STAG2. STAG2 correction alters thousands of individual chromatin loops (many gene-proximal) without affecting A/B compartments or TADs. Loops specific to STAG2-mutant cells (governed by STAG1-cohesin) are very large, consistent with greater loop extrusion processivity for STAG1-cohesin vs. STAG2-cohesin. STAG2 mutation activates Polycomb activity, increasing H3K27me3 marks. Endogenous STAG2 mutation correction by AAV-mediated targeting in two GBM cell lines; RNA-seq; Hi-C; ChIP-seq for H3K27me3 The Journal of biological chemistry High 38705393
2023 STAG2 inactivation in BRAF-mutant thyroid cancer cells decreases c-Myc protein stability via the ERK/AKT/GSK3β feedback pathway, thereby impairing glutamine metabolism by downregulating c-Myc targets SCL1A5, GLS, and GLS2, and conferring sensitivity to glutaminase inhibitor BPTES. STAG2 knockdown in BRAF-mutant thyroid cancer cell lines; c-Myc stability assays; ERK/AKT/GSK3β pathway analysis; glutamine deprivation and BPTES sensitivity assays; in vivo xenograft assays Cell death & disease Medium 37479689
2017 STAG2 loss promotes telomere recombination as an alternative mechanism of telomere maintenance. Despite loss of centromere cohesion, STAG2-mutant tumor cells maintain cohesion at chromosome arms and telomeres. STAG2 silencing in normal human telomerase-negative cells leads to increased telomere recombination, delayed telomere shortening, and postponed senescence onset. STAG2 silencing (shRNA and CRISPR) in normal human fibroblasts and tumor cell lines; telomere FISH; sister chromatid exchange assays at telomeres; telomerase inhibitor sensitivity; senescence assays Cancer research Medium 28819029

Source papers

Stage 0 corpus · 100 papers · ranked by NIH iCite citations
Year Title Journal Citations PMID
2014 Genomic landscape of Ewing sarcoma defines an aggressive subtype with co-association of STAG2 and TP53 mutations. Cancer discovery 443 25223734
2005 Dissociation of cohesin from chromosome arms and loss of arm cohesion during early mitosis depends on phosphorylation of SA2. PLoS biology 367 15737063
2014 The genomic landscape of the Ewing Sarcoma family of tumors reveals recurrent STAG2 mutation. PLoS genetics 361 25010205
2011 Mutational inactivation of STAG2 causes aneuploidy in human cancer. Science (New York, N.Y.) 361 21852505
2013 Recurrent inactivation of STAG2 in bladder cancer is not associated with aneuploidy. Nature genetics 214 24121791
2013 Frequent truncating mutations of STAG2 in bladder cancer. Nature genetics 169 24121789
2011 Specific sites in the C terminus of CTCF interact with the SA2 subunit of the cohesin complex and are required for cohesin-dependent insulation activity. Molecular and cellular biology 146 21444719
2009 Differential regulation of telomere and centromere cohesion by the Scc3 homologues SA1 and SA2, respectively, in human cells. The Journal of cell biology 140 19822671
2019 Cohesin Members Stag1 and Stag2 Display Distinct Roles in Chromatin Accessibility and Topological Control of HSC Self-Renewal and Differentiation. Cell stem cell 133 31495782
2018 Distinct roles of cohesin-SA1 and cohesin-SA2 in 3D chromosome organization. Nature structural & molecular biology 131 29867216
2021 STAG2 loss rewires oncogenic and developmental programs to promote metastasis in Ewing sarcoma. Cancer cell 99 34129824
2017 Synthetic lethality between the cohesin subunits STAG1 and STAG2 in diverse cancer contexts. eLife 94 28691904
2019 A requirement for STAG2 in replication fork progression creates a targetable synthetic lethality in cohesin-mutant cancers. Nature communications 88 30975996
2018 STAG2 deficiency induces interferon responses via cGAS-STING pathway and restricts virus infection. Nature communications 83 29662124
2019 Specific Contributions of Cohesin-SA1 and Cohesin-SA2 to TADs and Polycomb Domains in Embryonic Stem Cells. Cell reports 77 31216471
2013 Frequent inactivating mutations of STAG2 in bladder cancer are associated with low tumour grade and stage and inversely related to chromosomal copy number changes. Human molecular genetics 77 24270882
2021 STAG2 mutations alter CTCF-anchored loop extrusion, reduce cis-regulatory interactions and EWSR1-FLI1 activity in Ewing sarcoma. Cancer cell 76 33930311
2013 Distinct functions of human cohesin-SA1 and cohesin-SA2 in double-strand break repair. Molecular and cellular biology 75 24324008
2002 STAG2 and Rad21 mammalian mitotic cohesins are implicated in meiosis. EMBO reports 72 12034751
2013 Glioblastoma cells containing mutations in the cohesin component STAG2 are sensitive to PARP inhibition. Molecular cancer therapeutics 69 24356817
2016 Loss of cohesin complex components STAG2 or STAG3 confers resistance to BRAF inhibition in melanoma. Nature medicine 68 27500726
2015 A Phase 2 study of L-asparaginase encapsulated in erythrocytes in elderly patients with Philadelphia chromosome negative acute lymphoblastic leukemia: The GRASPALL/GRAALL-SA2-2008 study. American journal of hematology 65 26094614
2017 Synthetic lethal interaction between the tumour suppressor STAG2 and its paralog STAG1. Oncotarget 60 28430577
2017 Familial STAG2 germline mutation defines a new human cohesinopathy. NPJ genomic medicine 56 29263825
2020 Specialized functions of cohesins STAG1 and STAG2 in 3D genome architecture. Current opinion in genetics & development 52 32294612
1997 Purification of ribonucleases Sa, Sa2, and Sa3 after expression in Escherichia coli. Protein expression and purification 51 9367812
2015 Bioremediation potential of a highly mercury resistant bacterial strain Sphingobium SA2 isolated from contaminated soil. Chemosphere 49 26378869
2020 Cohesin SA1 and SA2 are RNA binding proteins that localize to RNA containing regions on DNA. Nucleic acids research 48 32352519
2017 De novo loss-of-function variants in STAG2 are associated with developmental delay, microcephaly, and congenital anomalies. American journal of medical genetics. Part A 48 28296084
2016 Intact Cohesion, Anaphase, and Chromosome Segregation in Human Cells Harboring Tumor-Derived Mutations in STAG2. PLoS genetics 46 26871722
2022 ASXL1 and STAG2 are common mutations in GATA2 deficiency patients with bone marrow disease and myelodysplastic syndrome. Blood advances 44 34529785
2022 STAG2 regulates interferon signaling in melanoma via enhancer loop reprogramming. Nature communications 38 35388001
2017 Cohesin SA2 is a sequence-independent DNA-binding protein that recognizes DNA replication and repair intermediates. The Journal of biological chemistry 38 29175904
2014 STAG2 promotes error correction in mitosis by regulating kinetochore-microtubule attachments. Journal of cell science 37 25074805
2008 Production of an extracellular thermohalophilic lipase from a moderately halophilic bacterium, Salinivibrio sp. strain SA-2. Journal of basic microbiology 36 18506896
2018 Mutations in STAG2 cause an X-linked cohesinopathy associated with undergrowth, developmental delay, and dysmorphia: Expanding the phenotype in males. Molecular genetics & genomic medicine 34 30447054
2020 Essential Roles of Cohesin STAG2 in Mouse Embryonic Development and Adult Tissue Homeostasis. Cell reports 33 32783938
2012 Mutational and expressional analyses of STAG2 gene in solid cancers. Neoplasma 31 22668012
2017 Loss of Tumor Suppressor STAG2 Promotes Telomere Recombination and Extends the Replicative Lifespan of Normal Human Cells. Cancer research 30 28819029
2015 Increased STAG2 dosage defines a novel cohesinopathy with intellectual disability and behavioral problems. Human molecular genetics 30 26443594
2020 STAG1 vulnerabilities for exploiting cohesin synthetic lethality in STAG2-deficient cancers. Life science alliance 29 32467316
2019 Hybrid Compound SA-2 is Neuroprotective in Animal Models of Retinal Ganglion Cell Death. Investigative ophthalmology & visual science 29 31348824
2021 STAG2 loss-of-function affects short-range genomic contacts and modulates the basal-luminal transcriptional program of bladder cancer cells. Nucleic acids research 27 34648034
2020 Chronic loss of STAG2 leads to altered chromatin structure contributing to de-regulated transcription in AML. Journal of translational medicine 27 32883299
2018 STAG2 Is a Biomarker for Prediction of Recurrence and Progression in Papillary Non-Muscle-Invasive Bladder Cancer. Clinical cancer research : an official journal of the American Association for Cancer Research 27 29954776
1995 ICP27 immediate early gene, glycoprotein K (gK) and DNA helicase homologues of infectious laryngotracheitis virus (gallid herpesvirus 1) SA-2 strain. Archives of virology 26 7794109
2014 STAG2 is a clinically relevant tumor suppressor in pancreatic ductal adenocarcinoma. Genome medicine 25 24484537
2019 Isolation and characterization of the Staphylococcus aureus bacteriophage vB_SauS_SA2. AIMS microbiology 22 31663062
2018 The role of STAG2 in bladder cancer. Pharmacological research 21 29501732
2015 Xq25 duplication: the crucial role of the STAG2 gene in this novel human cohesinopathy. Clinical genetics 21 25677961
2013 Xq25 duplications encompassing GRIA3 and STAG2 genes in two families convey recognizable X-linked intellectual disability with distinctive facial appearance. American journal of medical genetics. Part A 21 23637084
2019 STAG2 loss-of-function mutation induces PD-L1 expression in U2OS cells. Annals of translational medicine 19 31157248
2018 SA1/SA2 cohesion proteins and SIRT1-NAD+ deacetylase modulate telomere homeostasis in cumulus cells and are eligible biomarkers of ovarian aging. Human reproduction (Oxford, England) 19 29481647
2023 PAXIP1 and STAG2 converge to maintain 3D genome architecture and facilitate promoter/enhancer contacts to enable stress hormone-dependent transcription. Nucleic acids research 18 37070193
2015 Loss of STAG2 causes aneuploidy in normal human bladder cells. Genetics and molecular research : GMR 18 25867412
2022 STAG2 promotes the myelination transcriptional program in oligodendrocytes. eLife 17 35959892
2016 Antiviral activity of SA-2 against influenza A virus in vitro/vivo and its inhibition of RNA polymerase. Antiviral research 16 26802558
2024 Biologic and Clinical Analysis of Childhood Gamma Delta T-ALL Identifies LMO2/STAG2 Rearrangements as Extremely High Risk. Cancer discovery 15 38916500
2024 STAG2 mutations reshape the cohesin-structured spatial chromatin architecture to drive gene regulation in acute myeloid leukemia. Cell reports 15 39084219
2021 Paralogous synthetic lethality underlies genetic dependencies of the cancer-mutated gene STAG2. Life science alliance 15 34462321
2023 STAG2 Regulates Homologous Recombination Repair and Sensitivity to ATM Inhibition. Advanced science (Weinheim, Baden-Wurttemberg, Germany) 14 37985839
2021 Nanoencapsulated hybrid compound SA-2 with long-lasting intraocular pressure-lowering activity in rodent eyes. Molecular vision 14 33633438
2020 Loss of Stag2 cooperates with EWS-FLI1 to transform murine Mesenchymal stem cells. BMC cancer 14 31898537
2014 Microduplication of chromosome Xq25 encompassing STAG2 gene in a boy with intellectual disability. European journal of medical genetics 14 25450604
2020 Cohesin Components Stag1 and Stag2 Differentially Influence Haematopoietic Mesoderm Development in Zebrafish Embryos. Frontiers in cell and developmental biology 13 33365313
2009 Structure of RNase Sa2 complexes with mononucleotides--new aspects of catalytic reaction and substrate recognition. The FEBS journal 12 19558492
2024 STAG2: Computational Analysis of Missense Variants Involved in Disease. International journal of molecular sciences 11 38279279
2022 Modeling Down Syndrome Myeloid Leukemia by Sequential Introduction of GATA1 and STAG2 Mutations in Induced Pluripotent Stem Cells with Trisomy 21. Cells 11 35203280
2022 Neuroprotection of Rodent and Human Retinal Ganglion Cells In Vitro/Ex Vivo by the Hybrid Small Molecule SA-2. Cells 11 36497005
2015 C-terminus of Sororin interacts with SA2 and regulates sister chromatid cohesion. Cell cycle (Georgetown, Tex.) 11 25608232
2011 Familial skewed X-chromosome inactivation linked to a component of the cohesin complex, SA2. Journal of human genetics 11 21412246
2023 Modulation of Mitochondrial Metabolic Parameters and Antioxidant Enzymes in Healthy and Glaucomatous Trabecular Meshwork Cells with Hybrid Small Molecule SA-2. International journal of molecular sciences 10 37511316
2022 Somatic mosaicism in STAG2-associated cohesinopathies: Expansion of the genotypic and phenotypic spectrum. Frontiers in cell and developmental biology 10 36467423
2016 Complete genome sequence of 'Halanaeroarchaeum sulfurireducens' M27-SA2, a sulfur-reducing and acetate-oxidizing haloarchaeon from the deep-sea hypersaline anoxic lake Medee. Standards in genomic sciences 10 27182430
2016 The Expression of Cohesin Subunit SA2 Predicts Breast Cancer Survival. Applied immunohistochemistry & molecular morphology : AIMM 9 26447899
2016 Cohesin subunits, STAG1 and STAG2, and cohesin regulatory factor, PDS5b, in oral squamous cells carcinomas. Journal of oral pathology & medicine : official publication of the International Association of Oral Pathologists and the American Academy of Oral Pathology 9 27341316
2014 Penam sulfones and β-lactamase inhibition: SA2-13 and the importance of the C2 side chain length and composition. PloS one 9 24454944
2023 STAG2 inactivation reprograms glutamine metabolism of BRAF-mutant thyroid cancer cells. Cell death & disease 8 37479689
2022 STAG2 expression is associated with adverse survival outcomes and regulates cell phenotype in muscle-invasive bladder cancer. Cancer research communications 8 36275363
2024 Circular STAG2 RNA Modulates Bladder Cancer Progression via miR-145-5p/TAGLN2 and Is Considered as a Biomarker for Recurrence. Cancers 7 38473339
2022 Non-Invasive Detection of a De Novo Frameshift Variant of STAG2 in a Female Fetus: Escape Genes Influence the Manifestation of X-Linked Diseases in Females. Journal of clinical medicine 7 35887945
2012 Nuclear import and export signals of human cohesins SA1/STAG1 and SA2/STAG2 expressed in Saccharomyces cerevisiae. PloS one 7 22715410
2024 STAG2 loss in Ewing sarcoma alters enhancer-promoter contacts dependent and independent of EWS::FLI1. EMBO reports 6 39487368
2013 Aneuploidy in neuroblastoma tumors is not associated with inactivating point mutations in the STAG2 gene. BMC medical genetics 6 24088605
2024 STAG2 mutations regulate 3D genome organization, chromatin loops, and Polycomb signaling in glioblastoma multiforme. The Journal of biological chemistry 5 38705393
2024 Untangling the loops of STAG2 mutations in myelodysplastic syndrome. Leukemia & lymphoma 5 39264305
2022 STAG2 microduplication in a patient with eyelid myoclonia and absences and a review of EMA-related reported genes. European journal of medical genetics 5 36216271
2020 Persistent STAG2 mutation despite multimodal therapy in recurrent pediatric glioblastoma. NPJ genomic medicine 5 32528726
2013 STAG2 expression in oral cancer and potentially malignant lesions. Tumour biology : the journal of the International Society for Oncodevelopmental Biology and Medicine 5 24318971
2024 Emerging roles of cohesin-STAG2 in cancer. Oncogene 4 39613934
2023 Post-fusion influenza vaccine adjuvanted with SA-2 confers heterologous protection via Th1-polarized, non-neutralizing antibody responses. Vaccine 4 37330368
2014 STAG2 loss of expression is rare in aneuploid malignant salivary gland neoplasms. Journal of oral pathology & medicine : official publication of the International Association of Oral Pathologists and the American Academy of Oral Pathology 4 24822266
2004 Crystal structure reveals two alternative conformations in the active site of ribonuclease Sa2. Acta crystallographica. Section D, Biological crystallography 4 15213380
2025 Adhesin Component Member STAG2 Enhances Cisplatin Tolerance in Colorectal Cancer Cells through the Epithelial-mesenchymal Transition Pathway. Recent patents on anti-cancer drug discovery 3 38963118
2022 An active domain SA-2 derived from cystatin-SA, and its antifungal activity. Amino acids 3 36333524
2020 Solvent effect on excited state potential energy surfaces of Thioflavin T. Qualitatively different results by TDDFT and SA-2-CASSCF methods. Journal of computational chemistry 3 32516484
1996 Comparison of the genomic short regions of a vaccine strain (SA-2) and a virulent strain (CSW-1) of infectious laryngotracheitis virus (Gallid herpesvirus 1). Avian diseases 3 8713027
2025 Somatic mutations in STAG2 are associated with separated megakaryocyte nuclear lobes in myelodysplastic syndromes. Blood advances 2 40720765
2024 A STAG2-PAXIP1/PAGR1 axis suppresses lung tumorigenesis. The Journal of experimental medicine 2 39652422
2023 Identification of STAG2-Mutant Bladder Cancers by Immunohistochemistry. Methods in molecular biology (Clifton, N.J.) 2 37410232

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