Affinage

FXR1

RNA-binding protein FXR1 · UniProt P51114

Length
621 aa
Mass
69.7 kDa
Annotated
2026-06-09
89 papers in source corpus 38 papers cited in narrative 37 extracted findings
Cross-family judge vs UniProt: Affinage preferred faithfulness: 9/9 claims corpus-supported (100%)

Mechanistic narrative

Synthesis pass · prose summary of the discoveries below

FXR1 is a cytoplasmic RNA-binding protein of the Fragile X family that controls the stability, localization, and translation of target mRNAs to direct tissue-specific programs in muscle, brain, germline, and proliferating cells (PMID:7489725, PMID:7781595, PMID:15128702). It contains tandem KH domains and an RGG box that bind RNA, recognizes trimethylated peptides through N-terminal tandem Tudor domains, and forms heteromeric complexes with FMR1 and FXR2 while co-sedimenting with the 60S ribosomal subunit, consistent with a role in translational control (PMID:7489725, PMID:7781595, PMID:9259278, PMID:21072162). FXR1 engages diverse cis-elements—AU-rich elements and 3'/5'-UTR sequences as well as G-quadruplex structures—and its binding to G-quadruplex RNAs requires PRMT5-mediated arginine methylation of its nuclear export signal and RGG domain (PMID:17382880, PMID:34731628, PMID:38709899). Through these interactions it can either destabilize transcripts (e.g., p21/CDKN1A and pro-inflammatory mRNAs such as TNFα via HuR antagonism) or stabilize and translationally activate others (e.g., cMYC, where the RGG domain bridges eIF4A1 and eIF4E to circularize the mRNA) (PMID:27606879, PMID:30067974, PMID:34731628). FXR1 also assembles cytoplasm-spanning mRNP condensates: it packages exceptionally long mRNAs into a signaling scaffold required for RhoA-dependent actomyosin remodeling and binds cytoskeletal mRNAs together with actin, Arp2, and CYFIP1 to support smooth-muscle contractility and blood-pressure homeostasis (PMID:37043351, PMID:39106863). Liquid-liquid phase separation governed by an intrinsically disordered serine/arginine-rich region underlies tissue-specific functions, driving conversion of stored mRNAs to a translationally active state during spermiogenesis (PMID:32328638, PMID:35951695). Its activity is regulated by PAK1 phosphorylation at Ser420 and by an SCF(Fbxo4)/OTUD6B ubiquitination–deubiquitination balance that sets FXR1 protein levels (PMID:20417602, PMID:29142209, PMID:42056075). Recessive loss-of-function mutations in the muscle-specific exon-15 of FXR1 cause congenital multi-minicore myopathy, and exon-15 isoform choice controlled by Rbm24 governs myoblast proliferation versus fusion (PMID:30770808, PMID:39499773). In neurons FXR1 modulates parvalbumin-interneuron excitability via the T-type calcium channel Cav3.2 and controls homeostatic AMPA receptor scaling through a GSK3β-dependent mechanism (PMID:32893934, PMID:33863995).

Mechanistic history

Synthesis pass · year-by-year structured walk · 17 steps
  1. 1995 High

    Established FXR1 as a cytoplasmic RNA-binding protein and a member of a Fragile X family complex, defining its molecular identity and its physical partners.

    Evidence Co-immunoprecipitation, in vitro RNA binding, and subcellular fractionation across two independent reports

    PMID:7489725 PMID:7781595

    Open questions at the time
    • Functional consequence of FMR1/FXR2 heteromerization not defined
    • Specific RNA targets not identified
  2. 1997 Medium

    Linked FXR1 to the translation machinery by showing co-sedimentation with the 60S ribosomal subunit, implying a translational regulatory role.

    Evidence Sucrose gradient sedimentation / subcellular fractionation

    PMID:9259278

    Open questions at the time
    • Single method, single lab
    • Does not show whether association is direct or RNA-mediated
  3. 2004 High

    Demonstrated an essential physiological role in striated muscle, showing FXR1 is required for costamere organization and muscle integrity.

    Evidence Fxr1 knockout mouse with histochemistry and immunofluorescence of costameric proteins

    PMID:15128702

    Open questions at the time
    • Molecular targets mediating costamere defect not identified
    • Mechanism of perinatal lethality not resolved
  4. 2007 High

    Resolved how FXR1 regulates an ARE-bearing mRNA, showing it cooperates with AGO2 to activate rather than repress TNFα translation under specific conditions.

    Evidence In vivo crosslinking affinity purification, tethering, shRNA knockdown, reporter assays

    PMID:17382880

    Open questions at the time
    • Switch between activation and repression not mechanistically defined
    • Generality across other AREs unclear at the time
  5. 2010 High

    Defined post-translational and structural regulatory features: PAK1 phosphorylation at Ser420 required for muscle function, and tandem Tudor domains that read trimethylated peptides.

    Evidence In vitro kinase assay, mutagenesis, zebrafish rescue, X-ray crystallography with peptide-binding assays

    PMID:20417602 PMID:21072162

    Open questions at the time
    • Physiological methylated ligand of the Tudor domains not identified
    • Substrates/targets downstream of Ser420 phosphorylation not mapped
  6. 2013 High

    Established proteostatic control of FXR1 through SCF(Fbxo4)-mediated ubiquitination and a reciprocal feedback loop relevant to its overexpression in cancer.

    Evidence SCFFbxo4 affinity purification, co-IP, in vivo ubiquitination assay, Fbxo4 knockout analysis

    PMID:29142209

    Open questions at the time
    • Signals controlling Fbxo4 recognition of FXR1 not defined
    • Degron site on FXR1 not mapped
  7. 2016 Medium

    Identified G-quadruplex RNA recognition as a binding mode, linking FXR1 to p21 destabilization and telomerase maintenance in cancer.

    Evidence RIP, RNA pulldown, mRNA stability assay, G4 structure analysis in oral squamous carcinoma

    PMID:27606879

    Open questions at the time
    • Single lab
    • Determinants of G4 selectivity not yet defined
  8. 2017 High

    Extended FXR1 function to cardiac conduction and to chromatin-associated transcriptional regulation, including a collateral-lethality vulnerability in TP53/FXR2 co-deleted cancers.

    Evidence BioID, RIP, stability assays, cardiac KO/overexpression mice (#10); ChIP and proliferation assays in co-deleted lines (#11)

    PMID:28767039 PMID:29101288

    Open questions at the time
    • Mechanism of FXR1 recruitment to STAT-bound promoters unclear
    • How a cytoplasmic RNA-binding protein operates at chromatin not reconciled
  9. 2018 High

    Characterized FXR1 as an HuR antagonist that destabilizes pro-inflammatory mRNAs and mediates IL-19 anti-inflammatory signaling in vascular smooth muscle.

    Evidence LC-MS/MS interactome, RNA EMSA, RIP, knockdown/overexpression, stability assays

    PMID:30067974

    Open questions at the time
    • Competition mechanism with HuR not structurally resolved
    • Single lab/cell context
  10. 2019 High

    Provided human disease causation, defining exon-15 muscle isoform mutations as the cause of congenital multi-minicore myopathy, and uncovered amyloid-like FXR1 assemblies in healthy brain.

    Evidence Exome sequencing, conditional mouse models, histopathology (#13); amyloid-dye staining and proteomics of brain tissue (#30)

    PMID:30770808 PMID:31831836

    Open questions at the time
    • Molecular function of exon-15 isoforms in muscle not fully defined
    • Functional role of brain amyloid form not reconstituted
  11. 2020 High

    Established phase separation as a core organizing principle, with isoform- and splice-dependent condensate formation required for development and neuronal homeostasis controlled via GSK3β.

    Evidence In vitro condensate assays, Xenopus splice-specific morpholinos (#16); CRISPR KO, AMPA receptor assays, EEG, translatomics (#17)

    PMID:32328638 PMID:32893934

    Open questions at the time
    • Determinants linking specific condensates to specific mRNA fates not fully resolved
    • Mechanism of GSK3β-driven Fxr1 downregulation not detailed
  12. 2020 Medium

    Showed FXR1 protects bound small RNAs and target mRNAs from exoribonucleolytic decay, cooperating with miR301a-3p to silence p21.

    Evidence Small RNAseq, in vitro PNPT1 degradation protection assay, RIP, rescue

    PMID:31940341

    Open questions at the time
    • Single lang/cell context
    • General scope of miRNA stabilization not defined
  13. 2021 High

    Defined a translational-activation mechanism in which the RGG domain bridges eIF4A1/eIF4E to circularize and enhance cMYC translation, and a circuit controlling parvalbumin-interneuron excitability via Cav3.2.

    Evidence RIP, co-IP, SUnSET, 5'-cap pulldown (#19); PVI-specific KO, TRAP-seq, electrophysiology, rescue, behavior (#18)

    PMID:33863995 PMID:34731628

    Open questions at the time
    • Structural basis of eIF4 bridging not solved
    • Direct vs indirect regulation of Cav3.2 mRNA not fully distinguished
  14. 2022 High

    Demonstrated that LLPS converts stored mRNAs to a translationally active state during spermiogenesis, with a phase-separation-deficient knock-in reproducing the infertility phenotype, and a scaffolding role in 3'-end processing.

    Evidence Germline KO and L351P knock-in mice, LLPS assays, ribosome profiling (#20); co-IP, RIP, MS for CFIm recruitment (#21)

    PMID:35194031 PMID:35951695

    Open questions at the time
    • How phase separation mechanistically activates translation not fully resolved
    • CFIm scaffolding role tested in one cancer context
  15. 2023 Medium

    Connected FXR1 to cytoskeletal mRNA regulation and direct cytoskeletal protein binding underlying smooth-muscle contractility and blood pressure, and to cytoplasmic oxidative-stress/DNA-repair responses via MRE11.

    Evidence RIP-seq, MS, actin binding, conditional SMC KO with telemetry (#22); MRE11 MS/co-IP, fractionation, viability assays (#23)

    PMID:32211858 PMID:37043351

    Open questions at the time
    • MRE11 interaction is single-lab without reciprocal in vivo validation
    • Mechanism coupling cytoplasmic FXR1 to chromatin HR factors unclear
  16. 2024 High

    Resolved the cytoplasm-spanning mRNP signaling scaffold for RhoA-dependent actomyosin remodeling and the PRMT5-methylation requirement for G-quadruplex RNA binding, while expanding tissue and viral functions.

    Evidence Super-resolution imaging, fractionation, RhoA assays, FMR1-equivalent mutagenesis (#26); arginine mutagenesis, in vitro methylation, eCLIP (#25); BACE1 5'-UTR/ARL6IP1 (#24); exon-15/Rbm24 muscle splicing (#36); miR-1/Cx43 isoforms (#28); nuclear-pore condensate localization (#27); m6A reader in adenovirus (#33)

    PMID:36749074 PMID:37216506 PMID:38709899 PMID:39106863 PMID:39499773 PMID:40323739 PMID:41022782

    Open questions at the time
    • How methylation, phase separation, and scaffold assembly are integrated not unified
    • Several tissue/viral roles rest on single labs
  17. 2026 Medium

    Defined an OTUD6B deubiquitination feed-forward loop stabilizing FXR1 to drive MEK2/ERK signaling, and an MK5 splicing mechanism activating Wnt/β-catenin, positioning FXR1 as an oncogenic node.

    Evidence In vitro deubiquitination, co-IP, RIP, stability assays (#34); RNA-seq splicing, isoform assays, ASO model (#35)

    PMID:41954085 PMID:42056075

    Open questions at the time
    • Single-lab cancer-context findings
    • Generality across tumor types untested

Open questions

Synthesis pass · forward-looking unresolved questions
  • How the distinct FXR1 activities—RNA decay, translational activation, phase-separated scaffolding, splicing control, and amyloid/stress-granule assembly—are coordinated and selected within a single cell, and how nuclear-pore/TDP-43 functions relate to its cytoplasmic roles, remains unresolved.
  • No unified model linking condensate state to mRNA fate
  • Stress-granule/nuclear-pore findings rest on preprints or low-confidence assays (#31, #32, #29)

Mechanism profile

Synthesis pass · controlled-vocabulary classification · explore literature graph →
Molecular activity
GO:0003723 RNA binding 7 GO:0045182 translation regulator activity 4 GO:0060090 molecular adaptor activity 2 GO:0140098 catalytic activity, acting on RNA 2 GO:0008092 cytoskeletal protein binding 1 GO:0042393 histone binding 1
Localization
GO:0005829 cytosol 3 GO:0005635 nuclear envelope 1 GO:0005840 ribosome 1
Pathway
R-HSA-8953854 Metabolism of RNA 5 R-HSA-1266738 Developmental Biology 4 R-HSA-392499 Metabolism of proteins 3 R-HSA-162582 Signal Transduction 2 R-HSA-74160 Gene expression (Transcription) 1
Complex memberships
CFIm 3'-processing complexFMR1/FXR1/FXR2 heteromerSCF(Fbxo4) E3 ligase complex (substrate)

Evidence

Reading pass · 37 per-paper findings extracted from the source corpus
Year Finding Method Journal Conf PMIDs
1995 FXR1 protein contains two KH domains and an RGG box, binds RNA in vitro, and localizes to the cytoplasm; it forms heteromers with FMR1 and FXR2 both in vivo and in vitro, demonstrating that these three proteins interact as a family complex. Co-immunoprecipitation, in vitro RNA binding assay, subcellular fractionation/immunofluorescence The EMBO journal High 7489725 7781595
1997 FXR1 protein co-sediments with the 60S ribosomal subunit in cell fractionation experiments, consistent with a role in translational regulation. Sucrose gradient sedimentation / subcellular fractionation Human molecular genetics Medium 9259278
2004 Fxr1 knockout mice die shortly after birth with disrupted skeletal and cardiac muscle architecture; in wild-type neonates Fxr1p localizes to costameric regions of muscle, and its absence causes delocalization of costameric proteins vinculin, dystrophin, and alpha-actinin, establishing FXR1 as essential for striated muscle integrity. Knockout mouse model, histochemistry, immunofluorescence localization Human molecular genetics High 15128702
2007 FXR1 and AGO2 associate with the TNFα AU-rich element (ARE) exclusively during translation activation conditions (serum starvation); tethering and shRNA knockdown experiments demonstrate that both FXR1 and AGO2 are required for ARE-mediated translational upregulation of TNFα, and they are interdependent for this function. In vivo crosslinking-coupled affinity purification, tethering assay, shRNA knockdown, reporter assay Cell High 17382880
2010 PAK1 directly binds the KH2 domain of FXR1 via an 11-residue segment in PAK1's autoinhibitory domain; active PAK1 phosphorylates FXR1 at Ser420; the familial FMR1(I304N) mutation equivalent in FXR1 abolishes PAK1 binding; Ser420 phosphorylation is required for FXR1 function during zebrafish muscle development. Co-immunoprecipitation, in vitro kinase assay, phospho-specific antibodies, site-directed mutagenesis, zebrafish rescue experiments Molecular cell High 20417602
2010 X-ray crystal structures of the N-terminal tandem Tudor domains of FXR1 and FXR2 determined at 2.50 and 1.92 Å; these domains preferentially recognize trimethylated peptides in a sequence-specific manner, analogous to the tandem Tudor architecture of UHRF1. X-ray crystallography, biochemical peptide-binding assays PloS one High 21072162
2010 FXR1 loss-of-function in Xenopus laevis causes abnormal eye development and cranial neural crest cartilage defects; genetic interaction experiments link FXR1 function to the miRNA-induced silencing complex (RISC) pathway, including Dicer, during anterior neural development. Morpholino knockdown in Xenopus, epistasis with Dicer and specific miRNAs Developmental biology Medium 20197067
2013 Fbxo4 (SCF E3 ligase) directly ubiquitinates and degrades FXR1; purification of SCFFbxo4 complexes identified all three FXR family members as binding partners; FXR1 overexpression in turn attenuates Fbxo4 translation, creating a feedback loop that contributes to FXR1 overexpression in cancer. Affinity purification of SCFFbxo4, co-IP, in vivo ubiquitination assay, Fbxo4 knockout cell/tissue analysis Nature communications High 29142209
2014 Cytoplasmic plakophilins 1 and 3 (PKP1/3) bind FXR1 in an RNA-independent manner; this interaction is part of mRNP complexes that stabilize desmoplakin and PKP2 mRNAs, and both PKP3 and FXR1 are required for PKP2 mRNA stability. Co-immunoprecipitation (RNase-sensitive and -insensitive), gain/loss-of-function mRNA stability assays Molecular and cellular biology Medium 25225333
2016 FXR1 binds the G-quadruplex (G4) RNA structure in the 3′-UTR of p21 (CDKN1A) mRNA and promotes its degradation, thereby suppressing p21 protein expression; FXR1 also binds and stabilizes TERC RNA to maintain telomerase activity and bypass p53-mediated cellular senescence in oral squamous cell carcinoma. RNA immunoprecipitation, RNA pulldown, mRNA stability assay, shRNA knockdown, luciferase reporter, G4 structure analysis PLoS genetics Medium 27606879
2017 FXR1 associates with intercalated disc proteins and directly binds Cx43, Cx45, and ZO-1 mRNAs as identified by BioID proximity assay and RNA immunoprecipitation; FXR1 stabilizes these mRNAs and promotes their translation; cardiac-specific overexpression of FXR1 in mice redistributes gap junctions and induces ventricular tachycardia. BioID proximity assay, RNA immunoprecipitation, mRNA stability assay, luciferase reporter, cardiac-specific FXR1 knockout and AAV-overexpression mice Circulation High 29101288
2017 FXR1 recruits transcription factors STAT1 or STAT3 to gene promoters at the chromatin interface and regulates transcription; inhibition of FXR1 selectively blocks proliferation in cancer cells with homozygous deletion of both TP53 and FXR2 (collateral lethality). ChIP, chromatin association assays, siRNA knockdown, cell proliferation assays in TP53/FXR2 co-deleted cancer lines eLife Medium 28767039
2018 FXR1 is identified as an HuR-interacting protein in vascular smooth muscle cells (interaction is RNA-tethered, abrogated by RNase); FXR1 binds the ARE and 3′-UTR element of TNFα mRNA (RNA EMSA, RIP) and destabilizes pro-inflammatory mRNAs; FXR1 is induced by the anti-inflammatory cytokine IL-19 and is required for IL-19-mediated reduction of HuR and TNFα. LC-MS/MS interactome, RNA EMSA, RIP, siRNA knockdown and overexpression, mRNA stability assay Cell reports High 30067974
2019 Recessive loss-of-function mutations specifically in exon-15 of FXR1 (expressed only in cardiac and skeletal muscle isoforms) cause congenital multi-minicore myopathy in humans and mice; whole-body Fxr1 isoform depletion (Myf5-Cre) is neonatal lethal, whereas exon-15-specific mutations cause non-lethal myopathy. Whole-exome sequencing of patients, conditional mouse knockout models, histopathology Nature communications High 30770808
2019 FXR1 negatively regulates FBXO4 mRNA via direct association with its 3′-UTR, promoting mRNA degradation in prostate cancer cells; FBXO4 knockdown rescues the tumor-suppressive phenotype of FXR1-deficient cells. RNA immunoprecipitation, RNA pulldown, luciferase reporter, siRNA knockdown, epistasis rescue experiments Functional & integrative genomics Medium 30746571
2020 FXR1 controls stability of mature miR301a-3p in oral cancer cells; exoribonuclease PNPT1 degrades miR301a-3p when FXR1 is absent, and this degradation is blocked when FXR1-miRNA complex forms; FXR1 and miR301a-3p cooperatively target the 3′-UTR of p21 mRNA to promote its degradation. Small RNAseq, RNA stability assay, PNPT1 knockdown rescue, in vitro PNPT1 degradation assay, RIP PLoS genetics Medium 31940341
2020 FXR1 alternative splicing in its intrinsically disordered serine/arginine-rich domain produces isoforms with distinct capacities to form RNA-dependent biomolecular condensates in cells and in vitro; tissue-specific splicing of fxr1 is required for Xenopus development, and mis-splicing disrupts muscle condensate formation. RT-PCR isoform characterization, Xenopus loss-of-function with splice-specific morpholinos, in vitro condensate assays, cell-based condensate imaging The Journal of cell biology High 32328638
2020 Fxr1 is downregulated during synaptic scaling and sleep deprivation via a GSK3β-dependent mechanism; preventing Fxr1 downregulation during sleep deprivation blocks homeostatic modulation of surface AMPA receptors and synaptic strength; translatomic sequencing revealed Fxr1 contributes to sleep-deprivation-induced changes in neuronal translatomes. CRISPR/Cas9 somatic KO, gene overexpression, surface AMPA receptor assays, EEG recording, ribosome profiling/translatomics The EMBO journal High 32893934
2021 FXR1 deletion from parvalbumin interneurons (PVIs) of the medial prefrontal cortex reduces PVI excitability, impairs gamma oscillations, and causes schizophrenia-like behaviors; FXR1 regulates expression of the T-type calcium channel Cacna1h/Cav3.2 in PVIs; Cav3.2 inhibition phenocopies and Cav3.2 elevation rescues FXR1 deficiency behavioral deficits. PVI-specific Fxr1 conditional KO, translational profiling (TRAP), electrophysiology, AAV-mediated rescue, behavioral testing Molecular psychiatry High 33863995
2021 FXR1 binds AU-rich elements in the 3′-UTR of cMYC mRNA and stabilizes it; the RGG domain of FXR1 interacts with eIF4A1 and eIF4E; these dual interactions promote circularization of cMYC mRNA and recruitment of eukaryotic translation initiation factors to the translation start site, enhancing cMYC translation in ovarian cancer cells. RNA immunoprecipitation, co-immunoprecipitation, RPPA proteomics (SUnSET translation assay), mRNA stability assay, 5′-cap pulldown Cell reports Medium 34731628
2022 FXR1 undergoes liquid-liquid phase separation (LLPS) in late spermatids to merge mRNP granules with the translation machinery, converting stored mRNAs to a translationally active state; germline-specific Fxr1 ablation impairs target mRNA translation and causes male infertility; a phase-separation-deficient FXR1(L351P) knock-in produces the same developmental defect. Germline-specific conditional KO mice, FXR1-L351P knock-in mice, LLPS assays, ribosome profiling, immunofluorescence Science High 35951695
2022 FXR1 serves as a scaffold that recruits CFIm25 and CFIm68 to form a 3′-processing complex for sequence-specific poly(A) site recognition; FXR1 binding to TRAF1 mRNA leads to its nuclear stabilization and promotes cell proliferation in urothelial carcinoma. Co-immunoprecipitation, RNA immunoprecipitation, mass spectrometry, mRNA stability assay, knockdown functional assays Cell death & disease Medium 35194031
2023 FXR1 binds cytoskeletal mRNAs (identified by RIP-seq) and stabilizes them; FXR1 also directly binds actin and interacts with cytoskeletal proteins Arp2 and CYFIP1 (a WAVE regulatory complex protein); FXR1 depletion decreases adhesion, migration, contraction, and GTPase activation in VSMCs; conditional FXR1-SMC knockout mice show decreased blood pressure. RIP-seq, mass spectrometry, actin binding assay, siRNA knockdown functional assays, telemetry blood pressure measurement in conditional KO mice Cell reports High 37043351
2023 FXR1 interacts with MRE11 in the cytoplasm (partially at mitochondria); FXR1 depletion reduces oxidative stress responses and sensitizes cells to mitochondrial ROS inducer pyocyanin; FXR1 depletion also perturbs chromatin association of homologous recombination repair factors and sensitizes cells to camptothecin. Mass spectrometry identification of MRE11-binding partners, co-immunoprecipitation, subcellular fractionation, siRNA knockdown, cell viability assays Journal of radiation research Medium 32211858
2023 ARL6IP1 mediates FXR1 interaction with the 5′-UTR of BACE1 mRNA to regulate BACE1 translation; conophylline treatment promotes ARL6IP1-FXR1 interaction and inhibits FXR1 binding to the BACE1 5′-UTR both in vitro and in vivo, reducing BACE1 protein levels. RNA pulldown combined with LC-MS/MS, co-immunoprecipitation, 5′-UTR luciferase reporter, in vivo mouse (APP/PS1) model Proceedings of the National Academy of Sciences of the United States of America Medium 37216506
2024 PRMT5-mediated arginine methylation of FXR1 at R386, R388 (nuclear export signal) and R453, R455, R459 (RGG domain) is required for FXR1 binding to G-quadruplex (G4) RNA structures; PRMT5 loss-of-function inhibits FXR1 methylation and RNA-binding activity; eCLIP identifies G4-enriched mRNA targets including AHNAK, MAP1B, AHNAK2, HUWE1, DYNC1H1, and UBR4. Site-directed mutagenesis (point mutations of arginine residues), PRMT5 knockdown/inhibition, in vitro and in vivo methylation assay, eCLIP-seq, RNA binding assays with G4 disruption (LiCl) Nucleic acids research High 38709899
2024 FXR1 forms a cytoplasm-spanning mRNA-protein (mRNP) network by packaging exceptionally long mRNAs into condensate scaffolds; this FXR1 network functions as a signaling scaffold for RhoA-induced actomyosin reorganization by providing spatial proximity between kinases and their substrates; FMR1-equivalent point mutations in FXR1 disrupt the network and prevent actomyosin remodeling. Super-resolution microscopy, biochemical fractionation, RhoA signaling assays, site-directed mutagenesis (FMR1 disease-equivalent mutations), cell shape and migration assays Cell High 39106863
2024 Different RNA motifs (G-quadruplex-containing mRNAs vs. nucleoporin mRNAs) modulate formation of distinct FXR1 condensate subtypes; FXR1 interacts with nuclear pores and the opposing effects of different RNA motifs on FXR1 affinity for nuclear pores determine subcellular condensate localization; reduced FXR1 levels cause nuclear accumulation of transcribed RNAs and facilitate fate transition in human embryonic stem cells. Live-cell imaging, nuclear pore co-immunoprecipitation, RNA binding domain mutagenesis, RNA motif competition assays, hESC differentiation assays Nature communications Medium 41022782
2024 The muscle-specific isoforms FXR1-G and FXR1-E are required for miR-1-mediated repression of connexin 43 (Cx43) 3′-UTR; FXR1-G promotes physical interaction between miR-1 and the Cx43 3′-UTR; muscle-specific FXR1 isoforms are decreased in pediatric dilated cardiomyopathy left ventricular tissue. 3′-UTR luciferase reporter, co-immunoprecipitation of miR-1/FXR1/Cx43 mRNA complex, H9c2 differentiation model, Western blot of patient tissue American journal of physiology. Heart and circulatory physiology Medium 40323739
2025 SARS-CoV-2 NSP3 binds FXR1 via residues Y138 and F145 in the hypervariable region; NSP3-FXR1 interaction disrupts early stress granule formation; NSP3 Y138A/F145A mutant virus shows reduced replication in vitro and in vivo without altering interferon responses, attributed to loss of stress granule control. Reverse genetics mutant virus, in vitro binding assays, in vivo infection model, stress granule imaging bioRxivpreprint Medium bio_10.1101_2025.11.22.689758
2019 FXR1 protein forms amyloid-like aggregates (detergent-resistant, Congo-red birefringent, thioflavine-positive) in cortical neurons of healthy rat brain; RNA molecules co-localizing with FXR1 in these neurons are RNase A-insensitive, suggesting FXR1 functions in amyloid form in the brain. Immunoprecipitation from brain tissue, amyloid-specific dye staining (Congo red, Thioflavine S/T), proteomic approach for amyloid-like aggregate isolation Scientific reports Medium 31831836
2025 FXR1 colocalizes with core stress granule proteins (TIA-1, FMRP, FXR2, SFPQ) under heat shock and sodium arsenite stress in neuronal cells; in a yeast model, FXR1 physically interacts with these stress granule proteins; FXR1 co-localizes with anti-amyloid antibody OC under normal and stress conditions, suggesting amyloid FXR1-containing RNP particles interact with other stress granule components. Immunofluorescence co-localization, yeast two-hybrid/co-localization in yeast model, amyloid antibody staining Prion Low 40411539
2025 FXR1 loss in HAP1 cells causes nuclear pore pathology and passive egress of proteins and RNA from the nucleus; TDP-43 cytoplasmic mislocalization upon impaired nuclear import is restricted to FXR1 knockout cells (not FXR2 or FMR1 KO); loss of FXR1 specifically contributes to nuclear pore dysfunction-associated TDP-43 pathology. CRISPR KO of individual FXP family members, nuclear pore integrity assays, TDP-43 localization immunofluorescence, nuclear import impairment assays bioRxivpreprint Medium bio_10.1101_2025.10.27.684798
2025 FXR1 is an m6A reader protein in HAdV-5-infected cells; FXR1 controls stability of MLTU (major late transcription unit) mRNAs encoding viral capsid proteins; the long FXR1 isoform specifically interferes with MLTU mRNA translation; FXR1 depletion increases MLTU mRNA levels but paradoxically reduces capsid protein and infectious virus production. siRNA depletion, m6A-modified mRNA pulldown, mRNA stability assays, isoform-specific overexpression, plaque assays Journal of virology Medium 36749074
2026 OTUD6B deubiquitinase stabilizes FXR1 by removing K48-linked polyubiquitin chains via its catalytic activity; OTUD6B binds the KH domain of FXR1; FXR1 in turn binds and stabilizes MEK2 mRNA, activating ERK signaling; FXR1 also upregulates OTUD6B expression, creating a feed-forward oncogenic loop. Co-immunoprecipitation, in vitro deubiquitination assay, domain-mapping, mRNA stability assay, RIP, knockdown functional assays Cell death & disease Medium 42056075
2026 FXR1 drives alternative splicing of MK5 pre-mRNA to retain exon 6, generating a long kinase-competent isoform (MK5-L) that phosphorylates GSK3β and activates the Wnt/β-catenin pathway in hepatocellular carcinoma; ASO-mediated FXR1 targeting shifts splicing to the inactive MK5-S isoform and suppresses tumor growth. RNA-seq splicing analysis, isoform-specific overexpression/knockdown, in vitro/in vivo functional assays, ASO therapeutic model Cancer science Medium 41954085
2024 In skeletal muscle, exon-15-containing FXR1 isoform (Fxr1E15+) promotes myogenic differentiation and fusion while exon-15-lacking isoform (Fxr1E15−) regulates myoblast proliferation; increased inclusion of exon 15 during differentiation is mediated by binding of Rbm24 to the intron; the Fxr1E15+ isoform facilitates muscle regeneration in vivo. Isoform-specific knockdown in myoblasts, transcriptome and splicing analysis, Rbm24 splice-factor binding assay, in vivo muscle regeneration model Advanced science Medium 39499773

Source papers

Stage 0 corpus · 89 papers · ranked by NIH iCite citations
Year Title Journal Citations PMID
2007 AU-rich-element-mediated upregulation of translation by FXR1 and Argonaute 2. Cell 523 17382880
1995 The fragile X mental retardation syndrome protein interacts with novel homologs FXR1 and FXR2. The EMBO journal 272 7489725
1995 FXR1, an autosomal homolog of the fragile X mental retardation gene. The EMBO journal 206 7781595
1997 Differential expression of FMR1, FXR1 and FXR2 proteins in human brain and testis. Human molecular genetics 163 9259278
2022 LLPS of FXR1 drives spermiogenesis by activating translation of stored mRNAs. Science (New York, N.Y.) 134 35951695
2004 Fxr1 knockout mice show a striated muscle phenotype: implications for Fxr1p function in vivo. Human molecular genetics 110 15128702
2007 Frequent overexpression of the genes FXR1, CLAPM1 and EIF4G located on amplicon 3q26-27 in squamous cell carcinoma of the lung. International journal of cancer 92 17290396
2022 Exosomal circZNF451 restrains anti-PD1 treatment in lung adenocarcinoma via polarizing macrophages by complexing with TRIM56 and FXR1. Journal of experimental & clinical cancer research : CR 71 36209117
2001 Comparative genomic sequence analysis of the FXR gene family: FMR1, FXR1, and FXR2. Genomics 67 11735223
1995 Highly conserved 3' UTR and expression pattern of FXR1 points to a divergent gene regulation of FXR1 and FMR1. Human molecular genetics 62 8634689
2019 FXR1 promotes the malignant biological behavior of glioma cells via stabilizing MIR17HG. Journal of experimental & clinical cancer research : CR 60 30691465
2010 FMR1/FXR1 and the miRNA pathway are required for eye and neural crest development. Developmental biology 60 20197067
2017 Fbxo4-mediated degradation of Fxr1 suppresses tumorigenesis in head and neck squamous cell carcinoma. Nature communications 58 29142209
2016 RNA-Binding Protein FXR1 Regulates p21 and TERC RNA to Bypass p53-Mediated Cellular Senescence in OSCC. PLoS genetics 56 27606879
1999 Alternative splicing in the murine and human FXR1 genes. Genomics 55 10409431
2021 RNA-binding protein FXR1 drives cMYC translation by recruiting eIF4F complex to the translation start site. Cell reports 51 34731628
2010 A functional requirement for PAK1 binding to the KH(2) domain of the fragile X protein-related FXR1. Molecular cell 46 20417602
2010 Structural studies of the tandem Tudor domains of fragile X mental retardation related proteins FXR1 and FXR2. PloS one 45 21072162
2022 CircSETD3 mediates acquired resistance to gefitinib in non-small lung cancer cells by FXR1/ECT2 pathway. The international journal of biochemistry & cell biology 43 36503048
1999 Expression of FMR1, FXR1, and FXR2 genes in human prenatal tissues. Journal of neuropathology and experimental neurology 43 10446811
2020 FXR1 splicing is important for muscle development and biomolecular condensates in muscle cells. The Journal of cell biology 36 32328638
2024 The multifaceted role of Fragile X-Related Protein 1 (FXR1) in cellular processes: an updated review on cancer and clinical applications. Cell death & disease 35 38238286
2019 The RNA-binding protein FXR1 modulates prostate cancer progression by regulating FBXO4. Functional & integrative genomics 34 30746571
2018 FXR1 Is an IL-19-Responsive RNA-Binding Protein that Destabilizes Pro-inflammatory Transcripts in Vascular Smooth Muscle Cells. Cell reports 34 30067974
2019 Recessive mutations in muscle-specific isoforms of FXR1 cause congenital multi-minicore myopathy. Nature communications 33 30770808
2017 FXR1 regulates transcription and is required for growth of human cancer cells with TP53/FXR2 homozygous deletion. eLife 31 28767039
2017 Increased Cardiac Arrhythmogenesis Associated With Gap Junction Remodeling With Upregulation of RNA-Binding Protein FXR1. Circulation 31 29101288
2015 FXR1 is elevated in colorectal cancer and acts as an oncogene. Tumour biology : the journal of the International Society for Oncodevelopmental Biology and Medicine 31 26404134
2014 Plakophilins 1 and 3 bind to FXR1 and thereby influence the mRNA stability of desmosomal proteins. Molecular and cellular biology 31 25225333
2020 LncRNA TUG1 regulates ApoM to promote atherosclerosis progression through miR-92a/FXR1 axis. Journal of cellular and molecular medicine 30 32597038
2020 Circ_0000079 Decoys the RNA-Binding Protein FXR1 to Interrupt Formation of the FXR1/PRCKI Complex and Decline Their Mediated Cell Invasion and Drug Resistance in NSCLC. Cell transplantation 30 32951448
2021 FXR1 regulation of parvalbumin interneurons in the prefrontal cortex is critical for schizophrenia-like behaviors. Molecular psychiatry 29 33863995
2019 RNA-binding protein FXR1 is presented in rat brain in amyloid form. Scientific reports 29 31831836
2020 RNA binding protein FXR1-miR301a-3p axis contributes to p21WAF1 degradation in oral cancer. PLoS genetics 28 31940341
2020 Fxr1 regulates sleep and synaptic homeostasis. The EMBO journal 26 32893934
2018 Mental Illnesses-Associated Fxr1 and Its Negative Regulator Gsk3β Are Modulators of Anxiety and Glutamatergic Neurotransmission. Frontiers in molecular neuroscience 25 29706865
2021 circNRIP1 facilitates keloid progression via FXR1‑mediated upregulation of miR‑503‑3p and miR‑503‑5p. International journal of molecular medicine 23 33649815
2010 Fragile X-related protein FXR1 controls post-transcriptional suppression of lipopolysaccharide-induced tumour necrosis factor-alpha production by transforming growth factor-beta1. The FEBS journal 22 20491901
2012 Expression of RNA-binding proteins DND1 and FXR1 in the porcine ovary, and during oocyte maturation and early embryo development. Molecular reproduction and development 21 22730312
2022 FXR1 can bind with the CFIm25/CFIm68 complex and promote the progression of urothelial carcinoma of the bladder by stabilizing TRAF1 mRNA. Cell death & disease 20 35194031
2005 Two members of the Fxr gene family, Fmr1 and Fxr1, are differentially expressed in Xenopus tropicalis. The International journal of developmental biology 20 15968590
2016 Regulation of monocyte induced cell migration by the RNA binding protein, FXR1. Cell cycle (Georgetown, Tex.) 19 27229378
2004 Characterization of Fxr1 in Danio rerio; a simple vertebrate model to study costamere development. The Journal of experimental biology 18 15326209
2017 The interaction of GSK3B and FXR1 genotypes may influence the mania and depression dimensions in mood disorders. Journal of affective disorders 17 28242499
2024 The FXR1 network acts as a signaling scaffold for actomyosin remodeling. Cell 16 39106863
2024 FXR1 associates with and degrades PDZK1IP1 and ATOH8 mRNAs and promotes esophageal cancer progression. Biology direct 13 39511680
2022 The RNA-binding protein fragile-X mental retardation autosomal 1 (FXR1) modulates glioma cells sensitivity to temozolomide by regulating ferroptosis. Biochemical and biophysical research communications 13 35294905
2019 FXR1 expression domain in Wilms tumor. Journal of pediatric surgery 12 30894247
2023 ARL6IP1 mediates small-molecule-induced alleviation of Alzheimer pathology through FXR1-dependent BACE1 translation initiation. Proceedings of the National Academy of Sciences of the United States of America 11 37216506
2018 Muscle-Specific FXR1 Isoforms in Squamous Cell Cancer. Trends in cancer 11 30755307
2024 PRMT5-mediated arginine methylation of FXR1 is essential for RNA binding in cancer cells. Nucleic acids research 9 38709899
2023 FXR1 regulates vascular smooth muscle cell cytoskeleton, VSMC contractility, and blood pressure by multiple mechanisms. Cell reports 9 37043351
2022 CEP63 upregulates YAP1 to promote colorectal cancer progression through stabilizing RNA binding protein FXR1. Oncogene 9 35989368
2022 FXR1 promotes proliferation, invasion and migration of hepatocellular carcinoma in vitro and in vivo. Oncology letters 9 36466996
2021 Fragile X-Related Protein 1 (FXR1) Promotes Trophoblast Migration at Early Pregnancy via Downregulation of GDF-15 Expression. Reproductive sciences (Thousand Oaks, Calif.) 9 34291416
1998 The fragile-X-related gene FXR1 is a human autoantigen processed during apoptosis. The Journal of biological chemistry 9 9642279
2024 The Spatial-Temporal Alternative Splicing Profile Reveals the Functional Diversity of FXR1 Isoforms in Myogenesis. Advanced science (Weinheim, Baden-Wurttemberg, Germany) 8 39499773
2023 FXR1 facilitates axitinib resistance in clear cell renal cell carcinoma via regulating KEAP1/Nrf2 signaling pathway. Anti-cancer drugs 8 36730618
2020 FXR1 is a novel MRE11-binding partner and participates in oxidative stress responses. Journal of radiation research 8 32211858
2016 Correction: RNA-Binding Protein FXR1 Regulates p21 and TERC RNA to Bypass p53-Mediated Cellular Senescence in OSCC. PLoS genetics 8 27783624
2023 Fragile X-Related Protein FXR1 Controls Human Adenovirus Capsid mRNA Metabolism. Journal of virology 7 36749074
2023 Genetic Deletion of FXR1 Reduces Intimal Hyperplasia and Induces Senescence in Vascular Smooth Muscle Cells. The American journal of pathology 7 37080662
2018 Fragile X-related protein 1 (FXR1) regulates cyclooxygenase-2 (COX-2) expression at the maternal-fetal interface. Reproduction, fertility, and development 7 29852926
2025 Estrogen-induced FXR1 promotes endocrine resistance and bone metastasis in breast cancer via BCL2 and GPX4. Frontiers in cell and developmental biology 6 40196843
2024 Targeting KIFC1 Promotes Senescence in Soft Tissue Sarcoma via FXR1-Dependent Regulation of MAD2L1 mRNA Stability. Advanced science (Weinheim, Baden-Wurttemberg, Germany) 6 39387242
2021 Evidence of an interaction between FXR1 and GSK3β polymorphisms on levels of Negative Symptoms of Schizophrenia and their response to antipsychotics. European psychiatry : the journal of the Association of European Psychiatrists 6 33866994
2024 MicroRNA-320a enhances LRWD1 expression through the AGO2/FXR1-dependent pathway to affect cell behaviors and the oxidative stress response in human testicular embryonic carcinoma cells. Aging 5 38385979
2024 An in-depth review of the function of RNA-binding protein FXR1 in neurodevelopment. Cell and tissue research 5 39155323
2022 Amyloid Properties of the FXR1 Protein Are Conserved in Evolution of Vertebrates. International journal of molecular sciences 5 35887344
2023 FMRP, FXR1 protein and Dlg4 mRNA, which are associated with fragile X syndrome, are involved in the ubiquitin-proteasome system. Scientific reports 4 36732356
2022 Rg1 Promotes the Proliferation and Adipogenic Differentiation of Human Adipose-Derived Stem Cells via FXR1/Lnc-GAS5-AS1 Pathway. Current stem cell research & therapy 4 34844547
2022 FXR1-related congenital myopathy: expansion of the clinical and genetic spectrum. Journal of medical genetics 4 35393337
2018 Immunohistochemical Expression of FXR1 in Canine Normal Tissues and Melanomas. The journal of histochemistry and cytochemistry : official journal of the Histochemistry Society 4 29608406
2025 Dendrobium huoshanense polysaccharide inhibits NSCLC proliferation and immune evasion via FXR1-IL-35 axis signaling pathway. Journal of natural medicines 3 40259042
2024 FXR1 stabilizes SNORD63 to regulate blood-tumor barrier permeability through SNORD63 mediated 2'-O-methylation of POU6F1. International journal of biological macromolecules 3 38460644
2022 Over-expression of long non-coding RNA NORAD promotes trophoblastic cell viability, migration, and invasion in preeclampsia via the miR-202-5p/FXR1 axis. Taiwanese journal of obstetrics & gynecology 3 35361385
2025 Muscle-specific isoforms of FXR1 are necessary for miR-1-mediated repression of connexin 43 and are downregulated in pediatric dilated cardiomyopathy. American journal of physiology. Heart and circulatory physiology 1 40323739
2025 Functional amyloid protein FXR1 is recruited into neuronal stress granules. Prion 1 40411539
2025 Modular RNA interactions shape FXR1 condensates involved in mRNA localization and translation. Nature communications 1 41022782
2024 The FXR1 network acts as signaling scaffold for actomyosin remodeling. bioRxiv : the preprint server for biology 1 37961296
2023 FXR1 promotes glioma progression by downregulating microRNA-124-3p through long noncoding RNA FGD5-AS1 upregulation. Acta neurologica Belgica 1 37074635
2023 FXR1 impedes the development of osteoarthritis by targeting SND1. Clinical and experimental rheumatology 1 37083155
2021 Changes in FXR1 expression after Chemotherapy for Rhabdomyosarcoma. Journal of pediatric surgery 1 33736876
2021 Molecular and Immunohistochemical Expression of LTA4H and FXR1 in Canine Oral Melanoma. Frontiers in veterinary science 1 34966807
2026 circPLEKHM1 promotes tumor growth and metastasis through enhancing FXR1-mediated protein translation in PDAC. Cancer letters 0 41794277
2026 Single-cell transcriptomics reveals FXR1 as an actionable target for siRNA therapy in ovarian cancer. Nature communications 0 41932914
2026 FXR1-Directed Alternative Splicing of MK5 Drives Hepatocellular Carcinoma Progression by Activating GSK3β Signaling. Cancer science 0 41954085
2026 OTUD6B-mediated K48 deubiquitination of FXR1 forms a positive feedback loop activating MEK2/ERK signaling in colorectal cancer liver metastasis. Cell death & disease 0 42056075
2026 Contribution of FXR1 genetic polymorphisms to breast cancer susceptibility in Chinese females. Molecular genetics and genomics : MGG 0 42138731

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