Affinage

SFPQ

Splicing factor, proline- and glutamine-rich · UniProt P23246

Length
707 aa
Mass
76.1 kDa
Annotated
2026-06-10
100 papers in source corpus 41 papers cited in narrative 40 extracted findings
Cross-family judge vs UniProt: Affinage preferred faithfulness: 7/8 claims corpus-supported (88%)

Mechanistic narrative

Synthesis pass · prose summary of the discoveries below

SFPQ is a multifunctional nuclear DBHS protein that builds higher-order assemblies through an anti-parallel coiled-coil to template nuclear bodies and coordinate transcription, RNA processing, and genome maintenance (PMID:25765647, PMID:39698821). It dimerizes with itself and with related DBHS proteins NONO and PSPC1, with SFPQ/PSPC1 binding more tightly than SFPQ/NONO, establishing competitive partner selection within the family (PMID:29530979), and reaches a high-affinity, cooperative RNA-binding state through an induced-fit conformational switch at its domain-swapped RRM1-RRM2 interfaces (PMID:35998361). As a transcriptional regulator it acts both as a promoter-bound repressor that relocates to paraspeckles upon NEAT1 induction to de-repress IL8 during innate immune responses (PMID:24507715), and as an elongation factor that binds long introns and recruits CDK9 to sustain expression of long neuronal genes (PMID:29719248); it also suppresses TGF-β signaling by sequestering Smad4 in prion-like-domain-driven condensates (PMID:38103553). In RNA metabolism SFPQ enforces accurate splicing of long introns, suppresses cryptic last exons and premature polyadenylation (PMID:33476259, PMID:33771997), and partners with FUS to control Mapt exon 10 splicing and the 4R/3R-tau ratio (PMID:28147269). SFPQ assembles target mRNAs into axonal transport granules carried by the KIF5A/KLC1 kinesin motor, a function essential for axon survival (PMID:27019013, PMID:33284322). In genome maintenance the SFPQ•NONO complex binds internal DNA, drives end-independent synapsis and DNA-PKcs autophosphorylation to promote canonical NHEJ (PMID:27924002, PMID:25998385), supports homologous recombination via RAD51D (PMID:20813759), and suppresses telomeric R-loops while recruiting telomerase through hTR (PMID:30824709, PMID:40593584). SFPQ activity is tuned by PRMT1-mediated arginine methylation that enhances mRNA binding (PMID:25605962) and by zinc-induced polymerization that drives cytoplasmic aggregation (PMID:32034402). Aberrant cytoplasmic redistribution—through ALS-linked coiled-coil and zinc-center variants, an NLS-lacking alternatively spliced isoform, and self-propagating inclusions in Parkinson's disease—links SFPQ dysfunction to neurodegeneration (PMID:28392072, PMID:36168806, PMID:38761794, PMID:40845103).

Mechanistic history

Synthesis pass · year-by-year structured walk · 17 steps
  1. 2006 Medium

    Established that SFPQ acts together with the other DBHS proteins NONO and PSPC1 as a transcriptional coactivator, framing the family as cooperative partners rather than independent factors.

    Evidence Co-IP, luciferase reporters, and immunofluorescence in Sertoli cells showing reciprocal interaction and androgen receptor coactivation

    PMID:16641145

    Open questions at the time
    • No structural basis for heterodimer choice
    • Whether coactivation generalizes beyond androgen receptor unaddressed
  2. 2010 Medium

    Defined SFPQ as an active player in DNA double-strand break repair, distinguishing a direct HR role via RAD51D from rapid SFPQ/NONO recruitment to break sites.

    Evidence Co-IP with RAD51D, laser microirradiation recruitment imaging, HR/cohesion assays, and synthetic lethality testing in human cells

    PMID:20421735 PMID:20813759 PMID:21144806

    Open questions at the time
    • Mechanism distinguishing HR versus NHEJ contributions unresolved
    • N-terminal domain requirement for relocalization mechanistically undefined
  3. 2014 High

    Connected SFPQ promoter occupancy to lncRNA-controlled gene regulation, showing NEAT1-driven paraspeckle relocalization de-represses target genes in immunity, and identified competing RNA/protein partnerships (MALAT1, PTBP2).

    Evidence ChIP, NEAT1 induction, knockdown with IL8 readout, plus RIP/Co-IP characterizing MALAT1-SFPQ-PTBP2 in cancer cells

    PMID:24507715 PMID:25025966

    Open questions at the time
    • Generality of relocalization-based de-repression across promoters unknown
    • PTBP2 release mechanism is single-lab
  4. 2015 High

    Provided the structural and biochemical foundation: SFPQ polymerizes via an anti-parallel coiled-coil essential for cellular function, and the SFPQ•NONO complex performs end-independent DNA synapsis in NHEJ.

    Evidence Crystal structure, SAXS, EM, and mutagenesis with cellular readouts; in vitro DNA pairing and DNA-PKcs autophosphorylation assays with native complex

    PMID:25765647 PMID:25998385

    Open questions at the time
    • In vivo regulation of polymerization length not established
    • How synapsis activity integrates with canonical NHEJ machinery in cells partly open
  5. 2015 High

    Identified post-translational control of SFPQ RNA binding, showing PRMT1 arginine methylation enhances mRNA association and citrullination antagonizes it.

    Evidence Mass spectrometry of endogenous complex, in vitro PRMT1 methylation, and mRNP immunoprecipitation

    PMID:25605962

    Open questions at the time
    • Signals controlling methylation state in vivo unknown
    • Functional consequences for specific transcripts not mapped
  6. 2016 High

    Defined SFPQ as an RNA-granule organizer and elongation factor required for neuronal gene programs, linking it to axonal mRNA transport and cortical development.

    Evidence RIP/CLIP and axonal imaging in DRG neurons; CLIP-seq/ChIP-seq with conditional KO mouse for CDK9-dependent elongation; LSD1 Co-IP with in utero electroporation

    PMID:27019013 PMID:28034769 PMID:29719248

    Open questions at the time
    • How nuclear elongation and cytoplasmic transport functions are coordinated unclear
    • LSD1 pathway role is correlative
  7. 2017 High

    Established SFPQ's splicing function in disease-relevant contexts and confirmed a cytoplasmic requirement, showing FUS-SFPQ controls tau isoform ratio and that axonal SFPQ is functionally essential.

    Evidence In vivo hippocampal shRNA with rescue and behavior; zebrafish null rescue with cytoplasmic-only SFPQ and ALS variant analysis; Ago2 HITS-CLIP for nucleoplasmic miRNA targeting

    PMID:28147269 PMID:28392072 PMID:29084942

    Open questions at the time
    • Molecular basis of cytoplasmic SFPQ partner switching not resolved
    • Link between splicing and miRNA functions undefined
  8. 2017 High

    Reconstituted SFPQ•NONO substituting for XLF in canonical NHEJ in vitro while showing partly distinct cellular roles.

    Evidence In vitro end-joining, shRNA, radioresistance, and DNA-PKcs autophosphorylation assays

    PMID:27924002

    Open questions at the time
    • Relative in vivo contribution versus XLF not quantified
    • Single-lab reconstitution
  9. 2018 Medium

    Refined DBHS partner selection and identified an adaptor for SFPQ splicing utilization, showing SFPQ/PSPC1 outcompetes SFPQ/NONO and Dido3 recruits SFPQ to Pol II.

    Evidence Crystal structures with analytical ultracentrifugation; Dido3 KO mouse with Co-IP and RIP

    PMID:29530979 PMID:30931476

    Open questions at the time
    • Cellular determinants of which heterodimer forms not established
    • Whether Dido3 governs all SFPQ-dependent exons unknown
  10. 2019 Medium

    Extended genome-maintenance roles to telomeres and chromatin, showing SFPQ/NONO suppress telomeric R-loops and SFPQ-HDAC1 represses CD40 via histone modification switching.

    Evidence ChIP, DRIP, telomere FISH, knockdown for telomeres; ChIP/Co-IP and histone modification analysis for CD40

    PMID:30824709 PMID:31393052

    Open questions at the time
    • Mechanism coupling R-loop suppression to HR restraint partial
    • CD40 H3K36 switch is single-lab
  11. 2020 High

    Linked metal-driven polymerization to cytoplasmic aggregation and added m6A and cancer-splicing regulatory roles, mechanistically tying SFPQ biophysics to neurodegeneration.

    Evidence Zinc-bound crystal structure with neuron imaging; FTO photocrosslinking with m6A profiling; SFPQ/NONO-SRSF2 Co-IP/RIP in platinum-resistant ovarian cancer

    PMID:31981477 PMID:32034402 PMID:32332923

    Open questions at the time
    • Physiological zinc levels triggering aggregation in vivo unknown
    • FTO recruitment specificity determinants undefined
  12. 2021 High

    Identified the kinesin motor for axonal SFPQ-RNA granule transport and connected SFPQ to C9orf72 repeat RNA biology, broadening its neurodegeneration links.

    Evidence Reciprocal Co-IP, live transport imaging, mouse model, and CMT KIF5A mutation analysis; FISH/DPR quantification with overexpression and patient-derived cells

    PMID:33284322 PMID:33495278

    Open questions at the time
    • How motor cargo loading is regulated unknown
    • Mechanism by which SFPQ promotes repeat RNA foci not defined
  13. 2022 High

    Resolved the structural mechanism of cooperative RNA recognition and the SFPQ-NONO heterodimer architecture, mapping cancer mutations to the NOPS interface.

    Evidence Crystal structures of apo and RNA-bound SFPQ and of the SFPQ-NONO heterodimer

    PMID:35245601 PMID:35998361

    Open questions at the time
    • Functional consequence of NOPS-region cancer mutations untested
    • Cooperativity in the cellular context not validated
  14. 2022 High

    Connected familial ALS variants to enhanced zinc binding and a synaptic deficit, providing a structural mechanism for SFPQ aggregation in disease.

    Evidence Crystal structure of L534I, ITC zinc-binding affinity, primary neuron imaging, and GluA1/GluA2 surface quantification

    PMID:36168806

    Open questions at the time
    • In vivo pathogenicity of variants not demonstrated
    • Link from aggregation to GluA1 reduction mechanistically incomplete
  15. 2023 High

    Established phase separation as a regulatory mode, showing SFPQ condensates sequester Smad4 to suppress TGF-β and that the two low-complexity regions oppositely tune condensation.

    Evidence LLPS assays, Co-IP, ChIP, reporter, and KO for Smad4 sequestration; in vitro condensation and FRAP with LCR-deletion constructs

    PMID:37952770 PMID:38103553

    Open questions at the time
    • How condensate formation is triggered by upstream signals unclear
    • Relationship between Smad4 sequestration and paraspeckle condensates undefined
  16. 2024 High

    Expanded SFPQ into miRNA stability, viral life cycles, and a self-propagating neurodegenerative inclusion mechanism, diversifying its RNA-protective and pathological roles.

    Evidence BioID/small RNA-seq for miR-17~92 protection; CRISPR screen/KO and H1 ChIP for EBV latency; Co-IP/integration assay for HIV-1 integrase; patient brain fractionation and iPSC dopamine neurons for A-to-I-edited RNA inclusions

    PMID:38373651 PMID:38755141 PMID:38761794 PMID:39250314

    Open questions at the time
    • Whether viral and miRNA roles share a common biochemical basis unknown
    • Trigger initiating PD inclusion seeding undefined
  17. 2025 Medium

    Refined polymerization mechanics, telomerase recruitment, and the ALS redistribution mechanism, showing coiled-coil plasticity drives tetramerization and an NLS-lacking isoform underlies cytoplasmic SFPQ.

    Evidence Crystallography/SAXS of SFPQ/NONO coiled-coil variants; hTR Co-IP with telomere length assays; RT-PCR/fractionation/proteomics of altSFPQ in ALS models

    PMID:39698821 PMID:40593584 PMID:40845103

    Open questions at the time
    • Regulation of altSFPQ splicing choice unknown
    • Telomerase recruitment role is single-lab and Co-IP-based

Open questions

Synthesis pass · forward-looking unresolved questions
  • How SFPQ's many functions are partitioned—which heterodimer, modification state, and condensate context dictate transcription versus splicing versus DNA repair versus axonal transport in a given cell—remains unresolved.
  • No unified model linking partner choice to functional output
  • Physiological switches between nuclear and cytoplasmic SFPQ pools undefined
  • Causal contribution of SFPQ mislocalization to human disease in vivo not established

Mechanism profile

Synthesis pass · controlled-vocabulary classification · explore literature graph →
Molecular activity
GO:0003723 RNA binding 4 GO:0140110 transcription regulator activity 4 GO:0003677 DNA binding 2 GO:0060090 molecular adaptor activity 2 GO:0140313 molecular sequestering activity 1
Localization
GO:0005829 cytosol 4 GO:0005634 nucleus 3 GO:0005654 nucleoplasm 3
Pathway
R-HSA-73894 DNA Repair 3 R-HSA-74160 Gene expression (Transcription) 3 R-HSA-8953854 Metabolism of RNA 3 R-HSA-9609507 Protein localization 2 R-HSA-162582 Signal Transduction 1
Complex memberships
SFPQ-KIF5A/KLC1 transport complexSFPQ/NONO heterodimerSFPQ/PSPC1 heterodimerparaspeckle

Evidence

Reading pass · 40 per-paper findings extracted from the source corpus
Year Finding Method Journal Conf PMIDs
2015 Crystal structure of human SFPQ reveals it forms an extended linear polymer (>265 Å long) via an anti-parallel coiled-coil domain. This polymerization is reversible in solution and can be templated by DNA. Disruptive mutation of the coiled-coil interaction motif causes SFPQ mislocalization, reduced nuclear body formation, abrogated molecular interactions, and deficient transcriptional regulation, demonstrating that polymerization is essential for SFPQ cellular function. X-ray crystallography, small-angle X-ray scattering (SAXS), transmission electron microscopy, site-directed mutagenesis, cellular localization assays Nucleic acids research High 25765647
2014 SFPQ acts as a transcriptional repressor of IL8 by binding its promoter. Upon induction of lncRNA NEAT1 by viral infection or TLR3-p38 pathway stimulation, SFPQ is relocalized from the IL8 promoter to paraspeckles, thereby de-repressing IL8 transcription as part of the innate immune response. Chromatin immunoprecipitation, siRNA knockdown, NEAT1 overexpression/induction, immunofluorescence, reporter assays Molecular cell High 24507715
2018 SFPQ is required for transcriptional elongation of long genes (>100 kb) by RNA polymerase II. SFPQ co-transcriptionally binds long introns and mediates interaction of CDK9 with the elongation complex. Loss of SFPQ in developing mouse brains causes specific downregulation of long neuronal genes and neuronal apoptosis. CLIP-seq, ChIP-seq, mouse conditional knockout, RNA-seq, co-immunoprecipitation Cell reports High 29719248
2016 SFPQ functions as an RNA-binding protein that orchestrates an RNA regulon in dorsal root ganglion neurons, binding and co-assembling LaminB2 (Lmnb2) and Bclw (Bcl2l2) mRNAs into RNA granules and trafficking them to axons. SFPQ is required in nuclei, cytoplasm, and axons; its loss abolishes axonal transport of these mRNAs and causes neurotrophin-dependent axon degeneration. RIP, CLIP, fluorescence imaging, siRNA knockdown in neurons, in vivo mouse DRG experiments Nature neuroscience High 27019013
2021 SFPQ is required for accurate splicing of long introns and suppresses premature cleavage/polyadenylation events. SFPQ depletion leads to increased intron retention, cryptic splicing, premature transcription termination, and polyadenylation preferentially in long introns. SFPQ also suppresses the activation of cryptic last exons (CLEs) in neuronal genes. RNA-seq, SFPQ knockdown, bioinformatic analysis of splicing events across human, mouse, and zebrafish eLife High 33476259 33771997
2010 SFPQ (PSF) directly interacts with RAD51D and promotes homology-directed DNA repair. Deficiency of SFPQ alone causes sister chromatid cohesion defects and chromosome instability. SFPQ depletion delays DSB repair, and combined deficiency with RAD51D is synthetically lethal. Co-immunoprecipitation, siRNA knockdown, sister chromatid cohesion assay, DNA repair assays (comet, γH2AX) Nucleic acids research Medium 20813759
2010 The SFPQ/NONO heterodimer is rapidly recruited to laser-induced DNA double-strand break sites. SFPQ depletion delays DSB repair. MATR3 knockdown prolongs SFPQ/NONO retention at damage sites. Non-homologous end-joining proteins co-immunoprecipitate with NONO. Laser microbeam irradiation, live-cell imaging, siRNA knockdown, co-immunoprecipitation, cell cycle analysis Cell cycle (Georgetown, Tex.) Medium 20421735
2017 SFPQ•NONO complex promotes canonical non-homologous end joining (c-NHEJ) by substituting for XLF in vitro, promoting sequence-independent pairing of DNA substrates. In cell-based assays, NONO and XLF are both required for efficient end joining and radioresistance, indicating partly distinct functions. SFPQ•NONO complex also promotes DNA-PKcs autophosphorylation. In vitro end-joining assay, shRNA knockdown, radioresistance assay, DNA-PKcs autophosphorylation assay Nucleic acids research High 27924002
2015 The SFPQ•NONO complex binds DNA independently of free ends (unlike Ku), has DNA pairing/synapsis activity, and stimulates DNA-PKcs autophosphorylation. These activities support a model where SFPQ•NONO binds internal DNA sequences to stabilize a pre-ligation synaptic complex in NHEJ. Microwell-based DNA binding/pairing assay, in vitro DNA-PKcs autophosphorylation assay, native SFPQ•NONO purified from HeLa cells Biochemical and biophysical research communications High 25998385
2015 SFPQ is asymmetrically dimethylated at multiple arginines in its N-terminal domain by PRMT1. This arginine methylation increases SFPQ's association with mRNA in mRNP complexes. Citrullination of SFPQ antagonizes arginine methylation. Neither modification affects SFPQ/NONO complex formation. Mass spectrometry of endogenous complex, in vitro methylation assay with PRMT1, mRNP immunoprecipitation RNA (New York, N.Y.) High 25605962
2017 FUS and SFPQ interact in the nucleus to regulate alternative splicing of Mapt exon 10, controlling the ratio of 4-repeat tau (4R-T) to 3-repeat tau (3R-T). Hippocampus-specific knockdown of either FUS or SFPQ increases the 4R-T/3R-T ratio, causing FTLD-like behaviors, neuronal loss, and tau phosphorylation. Normalization of tau isoform ratio rescues the phenotype. Co-immunoprecipitation, in vivo shRNA knockdown in mouse hippocampus, RT-PCR for splice isoforms, behavioral testing Cell reports High 28147269
2021 SFPQ-RNA granules are transported anterogradely in axons by a tetrameric kinesin complex containing the motor KIF5A and adaptor KLC1. The SFPQ-KIF5A/KLC1 interaction is required for axon survival. KIF5A mutations causing Charcot-Marie-Tooth disease impair this binding. Co-immunoprecipitation, live imaging of RNA granule transport, genetic mouse model, CMT disease mutations The Journal of cell biology High 33284322
2017 SFPQ localizes to motor axons (in addition to nuclei) in zebrafish. Expression of a cytoplasmic-only SFPQ construct rescues motor axon defects and motility in sfpq null zebrafish. ALS-linked variants in the coiled-coil domain specifically impair axonal localization of SFPQ without rescuing axon morphology, demonstrating a functional requirement for axonal SFPQ. Zebrafish null mutant, rescue with cytoplasmic SFPQ construct, fluorescence imaging, ALS patient-derived variant analysis Neuron High 28392072
2019 SFPQ and NONO locate at telomeres and suppress RNA:DNA hybrid (R-loop) formation, telomere fragility, and homologous recombination at telomeres. SFPQ and NONO act as heterodimers in this function; their combined loss causes massive telomere recombination events in ALT cells. ChIP, R-loop detection (DRIP assay), telomere FISH, siRNA knockdown, co-immunoprecipitation Nature communications High 30824709
2020 SFPQ binds FTO (the m6A demethylase) directly via site-specific photocrosslinking. Overexpression of SFPQ promotes demethylation of m6A residues adjacent to SFPQ-binding sites across the transcriptome, indicating SFPQ recruits FTO to specific RNA targets to modulate their m6A status. Genetically encoded photocrosslinking, transcriptome-wide m6A profiling, SFPQ overexpression Cell chemical biology Medium 31981477
2017 Nucleoplasmic SFPQ interacts with Ago2 in an RNA-dependent manner and directly controls miRNA targeting of a subset of mRNAs by local binding. SFPQ binds long 3'-UTRs to form aggregates that optimize miRNA positioning/recruitment at selected binding sites, including let-7a binding to Lin28A 3'-UTR, extending miRNA-mediated silencing into the nucleoplasm. Quantitative proteomics, HITS-CLIP, transcriptomics, RNA immunoprecipitation, dual-luciferase reporter Nature communications High 29084942
2020 SFPQ in complex with p54nrb (NONO) binds and modulates SRSF2 splicing factor activity under platinum treatment in ovarian cancer cells. The SFPQ/NONO complex decreases SRSF2 binding to caspase-9 RNA, favoring expression of its antiapoptotic alternatively spliced form and promoting platinum resistance. Co-immunoprecipitation, RNA-IP, siRNA knockdown, alternative splicing analysis Oncogene Medium 32332923
2011 SFPQ (PSF) is an essential host factor for influenza A virus transcription. SFPQ knockdown reduces viral mRNA, cRNA, and vRNA accumulation >5-fold and specifically impairs polyadenylation of viral mRNAs without affecting cap-snatching, demonstrating SFPQ increases the efficiency of viral mRNA polyadenylation. siRNA knockdown, in vitro transcription of recombinant RNPs, virus yield assay, RNA accumulation analysis PLoS pathogens High 22114566
2010 PSF (SFPQ) contributes to radioresistance in human cells. The N-terminal domain of PSF is specifically required for both radioresistance and real-time relocalization of PSF-containing complexes to sites of laser-induced DNA damage. PSF, not its dimerization partner NONO, drives this relocalization. miRNA-mediated PSF knockdown, genetic rescue with PSF mutants, live-cell imaging of laser-induced DNA damage sites DNA repair Medium 21144806
2020 Zinc binding to SFPQ induces infinite polymerization via intermolecular interactions revealed by a 1.94 Å crystal structure. Application of zinc to primary cortical neurons induces cytoplasmic accumulation and aggregation of SFPQ. Mutagenesis of the three zinc-coordinating histidine residues reduces zinc-binding affinity and abolishes zinc-induced cytoplasmic aggregation. X-ray crystallography, site-directed mutagenesis, primary neuron imaging, zinc-binding affinity assay Nucleic acids research High 32034402
2022 Crystal structure of the SFPQ-NONO heterodimer resolved at 3.0 Å reveals subtle differences in the NOPS region orientation compared to other DBHS dimers, with amino acid substitutions in the NOPS region found mutated in aggressive cancers. X-ray crystallography Biochimie Medium 35245601
2018 Crystal structure of the SFPQ/PSPC1 heterodimer at 2.3 Å resolution. Analytical ultracentrifugation shows that SFPQ-containing heterodimers have higher affinity than the SFPQ homodimer, and the SFPQ/PSPC1 heterodimer has over 6-fold lower dissociation constant than SFPQ/NONO, suggesting PSPC1 can outcompete NONO for heterodimerization with SFPQ. X-ray crystallography, analytical ultracentrifugation The Journal of biological chemistry High 29530979
2019 Dido3 interacts with SFPQ via its carboxy terminus and recruits SFPQ to RNA polymerase II for alternative splicing. Loss of Dido3 suppresses SFPQ binding to RNA and increases exon skipping for SFPQ-dependent exons, identifying Dido3 as an adaptor that controls SFPQ utilization in RNA splicing. Co-immunoprecipitation, RNA-seq, SFPQ RIP, Dido3 knockout mouse model Nucleic acids research Medium 30931476
2014 MALAT1 lncRNA binds to SFPQ, releasing PTBP2 from the SFPQ/PTBP2 complex. The released PTBP2 promotes colorectal cancer cell proliferation and migration, identifying SFPQ as a repressor of PTBP2 function through direct complex formation. RNA immunoprecipitation, Co-immunoprecipitation, overexpression/knockdown experiments, in vivo xenograft British journal of cancer Medium 25025966
2006 SFPQ, NONO, and PSPC1 interact reciprocally in Sertoli cells and co-localize in androgen receptor complexes. All three DBHS proteins activate androgen receptor-mediated transcription in luciferase reporter assays in Sertoli cell lines. Co-immunoprecipitation, luciferase reporter assay, immunofluorescence in mouse testis Biology of reproduction Medium 16641145
2023 SFPQ suppresses TGF-β signaling through liquid-liquid phase separation (LLPS) mediated by its prion-like domain (PrLD). SFPQ physically sequesters Smad4 in its condensates, excluding Smad4 from the Smad complex and chromatin, thereby dampening Smad-dependent transcription. SFPQ deficiency or loss of phase separation activity renders cells hypersensitive to TGF-β. Co-immunoprecipitation, phase separation assays, ChIP, luciferase reporter, SFPQ knockout/knockdown Developmental cell High 38103553
2022 Crystal structure of PSF/SFPQ in complex with a 30-mer RNA at 3.5 Å reveals RNA recognition at two uracil-specific induced-fit binding pockets at domain-swapped inter-subunit RRM1-RRM2 interfaces. Apo structure lacks these pockets, indicating an all-or-nothing conformational transition to high-affinity RNA-binding state with positive cooperativity. X-ray crystallography (apo at 2.8 Å and RNA complex at 3.5 Å) Biochemistry High 35998361
2024 SFPQ supports EBV latency by maintaining expression of linker histone H1, which occupies latent EBV genomes including the BZLF1 immediate early gene promoter. SFPQ knockout triggers EBV lytic reactivation; enforced H1 expression rescues latency upon SFPQ KO, placing H1 downstream of SFPQ in the latency maintenance pathway. CRISPR/Cas9 screen, SFPQ KO, H1 ChIP, rescue by H1 overexpression, RT-PCR for viral gene expression Nature communications High 38755141
2022 Two familial ALS-linked SFPQ variants (N533H and L534I) near the zinc-coordinating centre have increased zinc-binding affinities. Crystal structure of SFPQ L534I at 1.83 Å reveals a second zinc-binding site. Overexpression of these mutants increases cytoplasmic SFPQ aggregates in primary neurons and reduces GluA1 (but not GluA2) AMPA receptor surface expression. Crystal structure, zinc-binding affinity assay (ITC), primary neuron imaging, surface GluA1/GluA2 quantification Open biology High 36168806
2023 The shorter C-terminal low-complexity region (LCR) of SFPQ is the main driver of condensate formation in vitro and in the nucleus. The longer N-terminal prion-like LCR attenuates condensation of the full-length protein, suggesting a regulatory role in preventing aberrant condensate formation. In vitro condensation assays, live-cell imaging with LCR deletion constructs, fluorescence recovery after photobleaching Journal of molecular biology Medium 37952770
2025 NONO, SFPQ, and PSPC1 associate with catalytically active telomerase through the hTR RNA component. Depletion of DBHS proteins causes telomerase retention in nuclear Cajal bodies and impairs telomerase recruitment to telomeres, resulting in progressive telomere shortening in multiple cell lines. Co-immunoprecipitation with hTR, telomerase activity assay, FISH for Cajal body retention, siRNA knockdown, telomere length measurement Nature communications Medium 40593584
2019 SFPQ interacts with HDAC1 and co-occupies the CD40 promoter in pulmonary adventitial fibroblasts, suppressing CD40 transcription by switching H3K36me3 to H3K36ac histone modification on the CD40 promoter region. ChIP assay, Co-immunoprecipitation, overexpression/knockdown, histone modification analysis Cell biology international Medium 31393052
2024 SFPQ depletion affects both primary and mature miRNA expression. SFPQ protects the oncogenic miR-17~92 polycistron from degradation by the nuclear NEXT-exosome complex. BioID on Drosha revealed proximity to SFPQ, and the effect is paraspeckle-independent (NEAT1 depletion does not replicate the SFPQ effect). BioID proximity ligation, SFPQ knockdown, transcriptomics, small RNA-seq, miR-17~92 stability assay Cell reports Medium 39250314
2024 In Parkinson's disease and dementia with Lewy bodies, NONO and SFPQ form novel nuclear inclusions with adenosine-to-inosine (A-to-I)-edited mRNAs. These NONO/SFPQ aggregates reduce expression of the editing inhibitor ADAR3, increasing A-to-I editing in axonal/synaptic transcripts. Inosine-containing transcripts bind more tightly to recombinant purified SFPQ in vitro and potentiate SFPQ aggregation in human dopamine neurons, creating a self-propagating pathological state. Biochemical fractionation of patient brain, in vitro RNA-binding assay with recombinant SFPQ, human iPSC-derived dopamine neurons, immunofluorescence Neuron High 38761794
2021 SFPQ overexpression increases numbers of both sense and antisense C9orf72 GGGGCC-repeat RNA foci and dipeptide repeat proteins (DPRs) in transfected cells. Conversely, SFPQ knockdown reduces RNA foci and DPRs in both transfected cells and patient-derived fibroblasts and lymphoblasts from C9orf72 mutation carriers. Overexpression/siRNA knockdown, FISH for RNA foci, DPR immunostaining in HEK cells and patient-derived cells Journal of cell science Medium 33495278
2025 SFPQ undergoes alternative splicing in ALS to produce an isoform (altSFPQ) lacking a nuclear localization sequence, leading to cytoplasm-predominant localization. altSFPQ has reduced phase separation potential and differential protein binding compared to canonical SFPQ. Increased altSFPQ transcript correlates with decreased canonical SFPQ in familial and sporadic ALS models, providing a mechanistic basis for SFPQ nuclear-to-cytoplasmic redistribution. RT-PCR, cellular fractionation, phase separation assays, proteomics, ALS patient/model analysis Science advances Medium 40845103
2024 SFPQ interacts with HIV-1 integrase (IN) via two diRGGX1-4 motifs in its N-terminal region. Single amino acid substitutions in either motif reduce IN binding, while mutations in both motifs almost completely disrupt interaction. SFPQ mutants with impaired IN binding fail to enhance viral integration, demonstrating SFPQ promotes HIV-1 integration through direct interaction with integrase. Co-immunoprecipitation, site-directed mutagenesis, viral integration assay Biochimie Medium 38373651
2025 Structural analysis of SFPQ/NONO heterodimer coiled-coil interactions reveals a flexible coiled-coil interface. Multiple coiled-coil interfaces (not just the canonical one) contribute to SFPQ/NONO tetramerization. A quadruple loss-of-function coiled-coil mutant (QM) abolishes tetramerization; a disulfide-forming variant (R542C) causes constitutive tetramerization, demonstrating the importance of coiled-coil plasticity for polymerization. X-ray crystallography, small-angle X-ray scattering with panel of SFPQ/NONO variants Nucleic acids research High 39698821
2016 SFPQ associates with LSD1 in the developing cerebral cortex. In vivo shRNA-mediated reduction of SFPQ impairs radial migration of newborn pyramidal neurons and decreases proliferation of progenitor cells, similar to LSD1/CoREST depletion, placing SFPQ in the LSD1/CoREST epigenetic regulatory pathway during cortical development. In utero electroporation of shRNA, Co-immunoprecipitation with LSD1, immunohistochemistry, migration assay International journal of developmental neuroscience Medium 28034769
2014 JNK1 interacts with SFPQ and NONO in an RNA-dependent manner in neuronal transport granules during NGF-induced differentiation. This interaction is specific (disrupted by JNK substrate-blocking peptide) and occurs in the cytosol. SFPQ knockdown inhibits neurite outgrowth in PC12 cells and acts in the same pathway as JNK. SILAC-based quantitative proteomics, co-immunoprecipitation, confocal microscopy, siRNA knockdown, neurite outgrowth assay Molecular & cellular proteomics : MCP Medium 25326457

Source papers

Stage 0 corpus · 100 papers · ranked by NIH iCite citations
Year Title Journal Citations PMID
2014 Long noncoding RNA NEAT1-dependent SFPQ relocation from promoter region to paraspeckle mediates IL8 expression upon immune stimuli. Molecular cell 560 24507715
2014 Long non-coding RNA MALAT1 promotes tumour growth and metastasis in colorectal cancer through binding to SFPQ and releasing oncogene PTBP2 from SFPQ/PTBP2 complex. British journal of cancer 327 25025966
2016 The DBHS proteins SFPQ, NONO and PSPC1: a multipurpose molecular scaffold. Nucleic acids research 271 27084935
2010 Involvement of Matrin 3 and SFPQ/NONO in the DNA damage response. Cell cycle (Georgetown, Tex.) 184 20421735
2015 The structure of human SFPQ reveals a coiled-coil mediated polymer essential for functional aggregation in gene regulation. Nucleic acids research 136 25765647
2016 The RNA-binding protein SFPQ orchestrates an RNA regulon to promote axon viability. Nature neuroscience 127 27019013
2018 Intron retention and nuclear loss of SFPQ are molecular hallmarks of ALS. Nature communications 125 29789581
2010 The splicing-factor related protein SFPQ/PSF interacts with RAD51D and is necessary for homology-directed repair and sister chromatid cohesion. Nucleic acids research 103 20813759
2014 Jak/Stat signaling stimulates zebrafish optic nerve regeneration and overcomes the inhibitory actions of Socs3 and Sfpq. The Journal of neuroscience : the official journal of the Society for Neuroscience 101 24523552
2019 SFPQ and NONO suppress RNA:DNA-hybrid-related telomere instability. Nature communications 99 30824709
2015 PSF/SFPQ is a very common gene fusion partner in TFE3 rearrangement-associated perivascular epithelioid cell tumors (PEComas) and melanotic Xp11 translocation renal cancers: clinicopathologic, immunohistochemical, and molecular characteristics suggesting classification as a distinct entity. The American journal of surgical pathology 96 26274027
2009 Perivascular epithelioid cell tumor with SFPQ/PSF-TFE3 gene fusion in a patient with advanced neuroblastoma. The American journal of surgical pathology 94 19606011
2017 Altered Tau Isoform Ratio Caused by Loss of FUS and SFPQ Function Leads to FTLD-like Phenotypes. Cell reports 90 28147269
2021 The RNA-binding protein SFPQ preserves long-intron splicing and regulates circRNA biogenesis in mammals. eLife 85 33476259
2012 Tau-mediated nuclear depletion and cytoplasmic accumulation of SFPQ in Alzheimer's and Pick's disease. PloS one 84 22558197
2017 SFPQ•NONO and XLF function separately and together to promote DNA double-strand break repair via canonical nonhomologous end joining. Nucleic acids research 81 27924002
2006 PSPC1, NONO, and SFPQ are expressed in mouse Sertoli cells and may function as coregulators of androgen receptor-mediated transcription. Biology of reproduction 79 16641145
2018 Loss of Sfpq Causes Long-Gene Transcriptopathy in the Brain. Cell reports 73 29719248
2011 The splicing factor proline-glutamine rich (SFPQ/PSF) is involved in influenza virus transcription. PLoS pathogens 72 22114566
2019 IGFBP-3 interacts with NONO and SFPQ in PARP-dependent DNA damage repair in triple-negative breast cancer. Cellular and molecular life sciences : CMLS 71 30725116
2017 Post-transcriptional gene silencing mediated by microRNAs is controlled by nucleoplasmic Sfpq. Nature communications 70 29084942
2020 The Emerging Role of the RNA-Binding Protein SFPQ in Neuronal Function and Neurodegeneration. International journal of molecular sciences 69 32998269
2020 SFPQ and Tau: critical factors contributing to rapid progression of Alzheimer's disease. Acta neuropathologica 66 32577828
2010 Sequences in PSF/SFPQ mediate radioresistance and recruitment of PSF/SFPQ-containing complexes to DNA damage sites in human cells. DNA repair 61 21144806
2020 Splicing factor proline- and glutamine-rich (SFPQ) protein regulates platinum response in ovarian cancer-modulating SRSF2 activity. Oncogene 60 32332923
2008 The complex of TFII-I, PARP1, and SFPQ proteins regulates the DYX1C1 gene implicated in neuronal migration and dyslexia. FASEB journal : official publication of the Federation of American Societies for Experimental Biology 59 18445785
2007 Whitesnake/sfpq is required for cell survival and neuronal development in the zebrafish. Developmental dynamics : an official publication of the American Association of Anatomists 58 17393485
2021 Binding and transport of SFPQ-RNA granules by KIF5A/KLC1 motors promotes axon survival. The Journal of cell biology 56 33284322
2017 Non-nuclear Pool of Splicing Factor SFPQ Regulates Axonal Transcripts Required for Normal Motor Development. Neuron 53 28392072
2015 Arginine methylation and citrullination of splicing factor proline- and glutamine-rich (SFPQ/PSF) regulates its association with mRNA. RNA (New York, N.Y.) 44 25605962
2008 The t(1;9)(p34;q34) and t(8;12)(p11;q15) fuse pre-mRNA processing proteins SFPQ (PSF) and CPSF6 to ABL and FGFR1. Genes, chromosomes & cancer 44 18205209
2022 Long noncoding RNA LHFPL3-AS2 suppresses metastasis of non-small cell lung cancer by interacting with SFPQ to regulate TXNIP expression. Cancer letters 40 35101541
2018 Crystal structure of a SFPQ/PSPC1 heterodimer provides insights into preferential heterodimerization of human DBHS family proteins. The Journal of biological chemistry 37 29530979
2020 SFPQ Is an FTO-Binding Protein that Facilitates the Demethylation Substrate Preference. Cell chemical biology 36 31981477
2020 SFPQ Depletion Is Synthetically Lethal with BRAFV600E in Colorectal Cancer Cells. Cell reports 32 32966782
2011 Chromatin remodeling as a mechanism for circadian prolactin transcription: rhythmic NONO and SFPQ recruitment to HLTF. FASEB journal : official publication of the Federation of American Societies for Experimental Biology 31 21507896
2022 Loss of NPPA-AS1 promotes heart regeneration by stabilizing SFPQ-NONO heteromer-induced DNA repair. Basic research in cardiology 30 35247074
2021 SFPQ promotes an oncogenic transcriptomic state in melanoma. Oncogene 30 34218270
2020 AR-induced long non-coding RNA LINC01503 facilitates proliferation and metastasis via the SFPQ-FOSL1 axis in nasopharyngeal carcinoma. Oncogene 30 32661324
2020 Aberrant interaction between FUS and SFPQ in neurons in a wide range of FTLD spectrum diseases. Brain : a journal of neurology 30 32770214
2023 Smad4 sequestered in SFPQ condensates prevents TGF-β tumor-suppressive signaling. Developmental cell 29 38103553
2018 NEAT1_2-SFPQ axis mediates cisplatin resistance in liver cancer cells in vitro. OncoTargets and therapy 29 30254462
2017 SFPQ/PSF-TFE3 renal cell carcinoma: a clinicopathologic study emphasizing extended morphology and reviewing the differences between SFPQ-TFE3 RCC and the corresponding mesenchymal neoplasm despite an identical gene fusion. Human pathology 29 28315422
2019 A novel human lncRNA SANT1 cis-regulates the expression of SLC47A2 by altering SFPQ/E2F1/HDAC1 binding to the promoter region in renal cell carcinoma. RNA biology 28 30951404
2015 Characterization of DNA binding and pairing activities associated with the native SFPQ·NONO DNA repair protein complex. Biochemical and biophysical research communications 28 25998385
2021 A conserved role for the ALS-linked splicing factor SFPQ in repression of pathogenic cryptic last exons. Nature communications 26 33771997
2020 Structural basis of the zinc-induced cytoplasmic aggregation of the RNA-binding protein SFPQ. Nucleic acids research 25 32034402
2018 MicroRNA-1296 Facilitates Proliferation, Migration And Invasion Of Colorectal Cancer Cells By Targeting SFPQ. Journal of Cancer 24 30026827
2024 Nuclear aggregates of NONO/SFPQ and A-to-I-edited RNA in Parkinson's disease and dementia with Lewy bodies. Neuron 23 38761794
2019 Loss of RNA-Binding Protein Sfpq Causes Long-Gene Transcriptopathy in Skeletal Muscle and Severe Muscle Mass Reduction with Metabolic Myopathy. iScience 23 30870781
2018 Dysregulation and Dislocation of SFPQ Disturbed DNA Organization in Alzheimer's Disease and Frontotemporal Dementia. Journal of Alzheimer's disease : JAD 22 29376859
2022 DNMT3A-mediated high expression of circ_0057504 promotes benzo[a]pyrene-induced DNA damage via the NONO-SFPQ complex in human bronchial epithelial cells. Environment international 20 36399942
2021 ALS/FTD-causing mutation in cyclin F causes the dysregulation of SFPQ. Human molecular genetics 20 33729478
2019 Dido3-dependent SFPQ recruitment maintains efficiency in mammalian alternative splicing. Nucleic acids research 20 30931476
2023 Different Low-complexity Regions of SFPQ Play Distinct Roles in the Formation of Biomolecular Condensates. Journal of molecular biology 19 37952770
2022 Crystal structure of SFPQ-NONO heterodimer. Biochimie 18 35245601
2020 SFPQ is involved in regulating arsenic-induced oxidative stress by interacting with the miRNA-induced silencing complexes. Environmental pollution (Barking, Essex : 1987) 18 32066060
2024 The nucleic acid binding protein SFPQ represses EBV lytic reactivation by promoting histone H1 expression. Nature communications 17 38755141
2022 Familial ALS-associated SFPQ variants promote the formation of SFPQ cytoplasmic aggregates in primary neurons. Open biology 17 36168806
2021 LncRNA NEAT1 Recruits SFPQ to Regulate MITF Splicing and Control RPE Cell Proliferation. Investigative ophthalmology & visual science 16 34787639
2014 Quantitative proteomics reveals dynamic interaction of c-Jun N-terminal kinase (JNK) with RNA transport granule proteins splicing factor proline- and glutamine-rich (Sfpq) and non-POU domain-containing octamer-binding protein (Nono) during neuronal differentiation. Molecular & cellular proteomics : MCP 16 25326457
2024 Established and Evolving Roles of the Multifunctional Non-POU Domain-Containing Octamer-Binding Protein (NonO) and Splicing Factor Proline- and Glutamine-Rich (SFPQ). Journal of developmental biology 15 38248868
2024 A novel protein encoded by circCOPA inhibits the malignant phenotype of glioblastoma cells and increases their sensitivity to temozolomide by disrupting the NONO-SFPQ complex. Cell death & disease 14 39183343
2022 Molecular Modelling of NONO and SFPQ Dimerization Process and RNA Recognition Mechanism. International journal of molecular sciences 14 35886974
2019 Exploitation of nuclear protein SFPQ by the encephalomyocarditis virus to facilitate its replication. Biochemical and biophysical research communications 14 30661786
2019 Amyloid precursor protein, an androgen-regulated gene, is targeted by RNA-binding protein PSF/SFPQ in neuronal cells. Genes to cells : devoted to molecular & cellular mechanisms 14 31541592
2025 GC-derived exosomal circMAN1A2 promotes cancer progression and suppresses T-cell antitumour immunity by inhibiting FBXW11-mediated SFPQ degradation. Journal of experimental & clinical cancer research : CR 13 39856764
2024 Follicular fluid exosome-derived miR-339-5p enhances in vitro maturation of porcine oocytes via targeting SFPQ, a regulator of the ERK1/2 pathway. Theriogenology 13 38805993
2023 Circular RNAs arising from synaptic host genes during human neuronal differentiation are modulated by SFPQ RNA-binding protein. BMC biology 13 37237280
2022 SFPQ Promotes Lung Cancer Malignancy via Regulation of CD44 v6 Expression. Frontiers in oncology 13 35707369
2019 A new crystal structure and small-angle X-ray scattering analysis of the homodimer of human SFPQ. Acta crystallographica. Section F, Structural biology communications 13 31204691
2024 KIF5A regulates axonal repair and time-dependent axonal transport of SFPQ granules and mitochondria in human motor neurons. Neurobiology of disease 12 39644980
2022 SFPQ-ABL1 and BCR-ABL1 use different signaling networks to drive B-cell acute lymphoblastic leukemia. Blood advances 12 35061886
2019 RNA-binding protein SFPQ cooperates with HDAC1 to suppress CD40 transcription in pulmonary adventitial fibroblasts. Cell biology international 12 31393052
2011 A tandem sequence motif acts as a distance-dependent enhancer in a set of genes involved in translation by binding the proteins NonO and SFPQ. BMC genomics 12 22185324
2022 Insight into the Tumor Suppression Mechanism from the Structure of Human Polypyrimidine Splicing Factor (PSF/SFPQ) Complexed with a 30mer RNA from Murine Virus-like 30S Transcript-1. Biochemistry 10 35998361
2025 M6A -mediated lncRNA SCIRT stability promotes NSCLC progression through binding to SFPQ and activating the PI3K/Akt pathway. Cellular and molecular life sciences : CMLS 9 39869159
2025 SFPQ::TFE3-rearranged PEComa: Differences and analogies with renal cell carcinoma carrying the same translocation. Pathology, research and practice 9 40239600
2024 Paraspeckle-independent co-transcriptional regulation of nuclear microRNA biogenesis by SFPQ. Cell reports 9 39250314
2023 SFPQ and Its Isoform as Potential Biomarker for Non-Small-Cell Lung Cancer. International journal of molecular sciences 9 37569873
2022 SFPQ promotes RAS-mutant cancer cell growth by modulating 5'-UTR mediated translational control of CK1α. NAR cancer 9 36177382
2020 Multilateral Bioinformatics Analyses Reveal the Function-Oriented Target Specificities and Recognition of the RNA-Binding Protein SFPQ. iScience 9 32659723
2017 SFPQ, a multifunctional nuclear protein, regulates the transcription of PDE3A. Bioscience reports 9 28743736
2025 Structural plasticity of the coiled-coil interactions in human SFPQ. Nucleic acids research 8 39698821
2025 A novel nuclear RNA HSD52 scaffolding NONO/SFPQ complex modulates DNA damage repair to facilitate temozolomide resistance. Neuro-oncology 7 39673809
2023 Primary Xp11 translocation PEComa of the testis with SFPQ⁃TFE3 rearrangement: a case report and review of the literature. Diagnostic pathology 7 36647137
2021 SFPQ regulates the accumulation of RNA foci and dipeptide repeat proteins from the expanded repeat mutation in C9orf72. Journal of cell science 7 33495278
2023 P-15 promotes chondrocyte proliferation in osteoarthritis by regulating SFPQ to target the Akt-RUNX2 axis. Journal of orthopaedic surgery and research 6 36915153
2019 Pigmented/melanocytic malignant perivascular epithelioid cell tumor with TFE3-SFPQ(PSF) rearrangement - a challenging diagnosis of PEComa family of tumors. Polish journal of pathology : official journal of the Polish Society of Pathologists 6 32146802
2016 SFPQ associates to LSD1 and regulates the migration of newborn pyramidal neurons in the developing cerebral cortex. International journal of developmental neuroscience : the official journal of the International Society for Developmental Neuroscience 6 28034769
2025 An engineered tumor organoid model reveals cellular identity and signaling trajectories underlying SFPQ-TFE3 driven translocation RCC. iScience 5 40463960
2025 SFPQ-TFE3 reciprocally regulates mTORC1 and induces lineage plasticity in a mouse model of renal tumorigenesis. Nature communications 5 41044182
2023 Induced pluripotent stem cell-derived extracellular vesicles overexpressing SFPQ protect retinal Müller cells against hypoxia-induced injury. Cell biology and toxicology 5 36790503
2022 [SFPQ and NONO Proteins and Long Non-Coding NEAT1 RNA: Cellular Functions and Role in the HIV-1 Life Cycle]. Molekuliarnaia biologiia 5 35403619
2021 [Aberrant Interaction Between FUS and SFPQ in Neurons in a Wide Range of FTLD Spectrum Diseases]. Brain and nerve = Shinkei kenkyu no shinpo 5 34462374
2024 The cellular SFPQ protein as a positive factor in the HIV-1 integration. Biochimie 4 38373651
2021 SFPQ associated with a co-activator for PPARγ, HELZ2, regulates key nuclear factors for adipocyte differentiation. Biochemical and biophysical research communications 4 34052659
2025 NONO, SFPQ, and PSPC1 promote telomerase recruitment to the telomere. Nature communications 3 40593584
2025 An alternative cytoplasmic SFPQ isoform with reduced phase separation potential is up-regulated in ALS. Science advances 3 40845103
2020 SFPQ-ABL1-positive B-cell precursor acute lymphoblastic leukemias. Genes, chromosomes & cancer 3 32306475

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