Affinage

MATR3

Matrin-3 · UniProt P43243

Round 2 corrected
Length
847 aa
Mass
94.6 kDa
Annotated
2026-04-28
73 papers in source corpus 20 papers cited in narrative 20 extracted findings

Mechanistic narrative

Synthesis pass · prose summary of the discoveries below

MATR3 is a nuclear matrix protein that functions as a multivalent RNA- and DNA-binding scaffold, organizing nuclear RNA processing, chromatin architecture, and gene expression through its two RNA recognition motifs (RRM1/RRM2). It anchors hyperedited inosine-containing RNAs to the nuclear matrix as part of a complex with p54nrb and PSF, represses cryptic exon inclusion in pre-mRNA splicing, inhibits primary miRNA processing, and forms phase-separated meshworks with antisense LINE1 RNAs to coordinate higher-order chromatin topology including intra-TAD interactions and H3K27me3 distribution (PMID:11525732, PMID:37381832, PMID:38320753, PMID:30790622). MATR3 additionally recruits m6A machinery components (METTL3–METTL14 complex, YTHDF2) to regulate mRNA stability of inflammatory and immune signaling transcripts, directly binds and inhibits the DUX4 transcription factor relevant to facioscapulohumeral muscular dystrophy, and controls translation of pluripotency transcripts at ribosomes (PMID:36368640, PMID:39276912, PMID:37703175, PMID:33733063). Disease-linked mutations differentially compromise MATR3 solubility and RNA-binding activity: the S85C variant causes loss of nuclear MATR3 and ALS-like motor neuron degeneration in knock-in mice, while the RRM2 M548T variant additionally abolishes RNA binding and cryptic splicing repression, linking MATR3 to ALS, distal myopathy, and neurodevelopmental disease (PMID:33082323, PMID:38320753). Perturbation of MATR3 expression levels also causes congenital cardiovascular outflow tract defects in mice (PMID:25574029).

Mechanistic history

Synthesis pass · year-by-year structured walk · 11 steps
  1. 2001 High

    Identification of MATR3 as a component of the p54nrb/PSF nuclear retention complex established its foundational role as a nuclear matrix anchor for hyperedited RNAs, answering how A-to-I-edited dsRNAs are prevented from nuclear export.

    Evidence Biochemical fractionation of HeLa nuclear extracts and RNA affinity purification with inosine-containing substrates

    PMID:11525732

    Open questions at the time
    • Which MATR3 domains mediate the interaction with p54nrb/PSF was not mapped
    • Whether MATR3 directly contacts RNA or acts solely as a scaffold in this complex was unresolved
  2. 2014 Medium

    Demonstration that both RRM domains of MATR3 contribute to selective small RNA binding, with phosphorylation enhancing affinity, established that MATR3's RNA-binding activity is regulatable post-translationally.

    Evidence Mass spectrometry-based RNA pull-down from retinal tissue with RRM domain mapping and phosphorylation assays

    PMID:24558381

    Open questions at the time
    • The specific kinase(s) responsible for enhancing RNA binding were not identified
    • Structural basis for phosphorylation-dependent RNA affinity changes was not resolved
  3. 2015 High

    A gene trap mouse model revealed that even subtle perturbation of MATR3 expression causes congenital left ventricular outflow tract defects, establishing dosage sensitivity in cardiac development.

    Evidence Matr3 Gt-ex13 gene trap mouse with cardiac phenotyping of heterozygotes

    PMID:25574029

    Open questions at the time
    • The downstream transcripts or splicing events affected in developing heart were not identified
    • Whether MATR3 acts through RNA processing or chromatin organization in cardiac morphogenesis is unknown
  4. 2018 Medium

    Binding of MATR3 to the lncRNA SNHG1 and its role as an inhibitor of pri-miR-138-2 processing extended the functional repertoire of MATR3 to non-coding RNA regulation, linking it to dendritic spine morphogenesis and neuroblastoma RNA splicing.

    Evidence RNA pull-down/LC-MS/MS and RIP for SNHG1; biochemical pull-down and loss-of-function for pri-miR-138 in HEK293 and primary neurons

    PMID:30516047 PMID:30790622

    Open questions at the time
    • Whether MATR3 inhibits pri-miRNA processing via steric occlusion or by blocking Drosha/DGCR8 was not determined
    • Functional consequences of the MATR3-SNHG1 interaction on specific splicing targets were not fully mapped
  5. 2020 High

    CRISPR knock-in of the ALS-linked S85C mutation in mice and transgenic Drosophila models collectively established that MATR3 mutations cause motor neuron degeneration through loss of nuclear MATR3 and increased protein insolubility, with axonal transport as a genetic modifier.

    Evidence S85C knock-in mouse with behavioral/neuropathological analysis; Drosophila motor neuron expression with modifier screen

    PMID:32515490 PMID:33082323

    Open questions at the time
    • Whether S85C toxicity is purely loss-of-function or involves a toxic gain-of-function from cytoplasmic aggregates was not resolved
    • The specific RNA targets lost upon S85C-driven MATR3 mislocalization were not identified
  6. 2020 High

    Discovery that MATR3 and PTBP1 bind pCharme lncRNA intron-1 to form nuclear aggregates required for chromatin domain organization during myogenesis established MATR3 as an organizer of lncRNA-dependent chromatin architecture.

    Evidence RAP-MS in myogenic cells; CRISPR intron-1 deletion in mouse with cardiac phenotyping

    PMID:33357424

    Open questions at the time
    • Whether MATR3 phase separation properties contribute to pCharme-dependent chromatin domain formation was not tested
    • The generality of this lncRNA-scaffolding role across tissues beyond muscle was unknown
  7. 2021 Medium

    Demonstration that MATR3 binds OCT4/YTHDF1 promoters and controls ribosomal translation of pluripotency transcripts revealed dual transcriptional and translational roles for MATR3 in stem cell maintenance.

    Evidence ChIP, ribosome fractionation, and RIP in human iPSCs after MATR3 knockdown

    PMID:33733063

    Open questions at the time
    • Whether MATR3 directly contacts ribosomes or is co-purified through mRNA tethering was not distinguished
    • Relevance to in vivo pluripotency maintenance was not tested
  8. 2022 Medium

    Identification of MATR3 as a regulator of the METTL3–METTL14 m6A methyltransferase complex in macrophages, suppressing inflammatory MAPK signaling through m6A-mediated mRNA decay, established MATR3 as a node linking epitranscriptomic regulation to innate immunity.

    Evidence Co-IP, m6A-seq, and MATR3 overexpression in mouse atherosclerosis model

    PMID:36368640

    Open questions at the time
    • Whether MATR3 directly promotes METTL3-METTL14 complex assembly or stabilizes it post-assembly was not determined
    • The RNA targets undergoing MATR3-dependent m6A modification in macrophages were not comprehensively catalogued
  9. 2023 High

    Multiple 2023 studies collectively established that MATR3 organizes higher-order chromatin through phase-separated meshworks with antisense LINE1 RNAs, directly inhibits DUX4 transcriptional activity relevant to FSHD, participates in the ADD1/ADAR1 RNA-editing axis, and represses cryptic exon inclusion—with disease mutations differentially affecting solubility, RNA binding, and splicing repression.

    Evidence Hi-C/ATAC-seq/phase-separation assays for LINE1 meshwork; Co-IP/domain mapping/FSHD cell rescue for DUX4; RIP/A-to-I editing assays for ADAR1 axis; RNA-seq/biochemistry for cryptic exon repression and mutant characterization

    PMID:37156877 PMID:37381832 PMID:37703175 PMID:38320753

    Open questions at the time
    • The structural basis for MATR3 phase separation and how ALS mutations alter condensate biophysics at the atomic level is unresolved
    • Whether cryptic exon repression and LINE1-dependent chromatin organization are mechanistically linked functions or independent
    • The full spectrum of cryptic exons regulated by MATR3 across neuronal subtypes is not known
  10. 2024 Medium

    MATR3 was shown to recruit the m6A reader YTHDF2 to DHX58 mRNA for degradation, suppressing type I IFN signaling in liver cancer and linking MATR3 to immune evasion, while parallel work connected MATR3-mediated REST mRNA regulation to UNC13A expression in motor neurons.

    Evidence RIP with RRM mutants and YTHDF2 Co-IP in liver cancer cells with in vivo knockout; RNA-binding and knockdown assays in motor neuron models (preprint)

    PMID:39276912 PMID:bio_10.1101_2024.10.22.619761

    Open questions at the time
    • The REST–UNC13A axis finding awaits peer review
    • Whether MATR3-YTHDF2 interaction is direct or mediated by the m6A-modified mRNA substrate was not resolved
    • How tumor cells upregulate MATR3 to achieve immune evasion is unknown
  11. 2025 Medium

    Studies in 2025 revealed that IFN-I-driven phosphorylation modulates MATR3's RNA-binding activity, that EZH2-mediated DDX1 methylation drives MATR3 exon 14 skipping to produce a truncated isoform disrupting nuclear architecture, and that MATR3 promotes ASFV replication through interaction with viral p30 protein.

    Evidence RNA interactome capture before/after IFN-I (preprint); splicing/ATAC-seq analysis of MATR3 truncation in disc degeneration model; Co-IP/knockdown with ASFV replication assay

    PMID:39919500 PMID:40610464 PMID:bio_10.1101_2025.05.30.656608

    Open questions at the time
    • The IFN-I phosphoregulation finding awaits peer review
    • Specific phosphosites mediating IFN-I-driven changes in MATR3 RNA binding are not fully mapped
    • Whether the truncated MATR3 isoform acts in a dominant-negative fashion in other tissues is unknown

Open questions

Synthesis pass · forward-looking unresolved questions
  • Key unresolved questions include the atomic-resolution structural basis for MATR3 phase separation and its regulation by disease mutations, the comprehensive identity of MATR3-repressed cryptic exons across neuronal and cardiac lineages, and whether MATR3's chromatin-organizing and splicing-repression functions are mechanistically coupled or independent.
  • No high-resolution structure of full-length MATR3 or its phase-separated state exists
  • Systematic identification of MATR3-dependent cryptic exons across tissues is incomplete
  • The mechanistic relationship between MATR3's RNA-anchoring, chromatin-organizing, and m6A-regulatory functions is undefined

Mechanism profile

Synthesis pass · controlled-vocabulary classification · explore literature graph →
Molecular activity
GO:0003723 RNA binding 7 GO:0098772 molecular function regulator activity 3 GO:0003677 DNA binding 2 GO:0140110 transcription regulator activity 2
Localization
GO:0005634 nucleus 8 GO:0005654 nucleoplasm 3 GO:0005694 chromosome 2
Pathway
R-HSA-8953854 Metabolism of RNA 4 R-HSA-1643685 Disease 3 R-HSA-168256 Immune System 2 R-HSA-4839726 Chromatin organization 2 R-HSA-74160 Gene expression (Transcription) 2
Partners
ADAR1DUX4METTL14METTL3NONOPTBP1SFPQYTHDF2
Complex memberships
METTL3-METTL14 methyltransferase complex (regulatory interaction)p54nrb/PSF/MATR3 nuclear retention complex

Evidence

Reading pass · 20 per-paper findings extracted from the source corpus
Year Finding Method Journal Conf PMIDs
2001 Matrin 3 (MATR3) is a component of a multiprotein nuclear complex that includes p54(nrb) and PSF, which binds specifically to inosine-containing (hyperedited) RNAs and anchors them to the nuclear matrix, preventing their export while allowing selectively edited mRNAs to be exported. Biochemical fractionation of HeLa nuclear extracts, RNA affinity purification, co-purification of complex components Cell High 11525732
2014 The small RNA pY RNA1-s2 selectively binds to Matrin 3 (Matr3) via both of its RNA recognition motifs (RRM1 and RRM2), with binding specificity dependent on RNA sequence and enhanced by phosphorylation of Matr3. Mass spectrometric analysis of RNA pull-down products from retinal tissue, followed by domain-mapping and phosphorylation assays PloS one Medium 24558381
2015 Disruption of the MATR3 3' UTR (affecting polyadenylation and protein expression) in mice via a gene trap allele causes congenital cardiovascular defects including bicuspid aortic valve, coarctation of the aorta, and patent ductus arteriosus, demonstrating that subtle perturbations in Matrin 3 expression levels are sufficient to cause left ventricular outflow tract defects. Mouse gene trap model (Matr3 Gt-ex13), RT-PCR/polyadenylation analysis, cardiac phenotyping of heterozygotes Human molecular genetics High 25574029
2019 Matrin 3 (Matr3) acts as an inhibitory regulator of pri-miR-138-2 processing in the nucleus; Matrin-3 physically interacts with the loop region of pri/pre-miR-138-2 and loss-of-function of Matrin-3 increases mature miR-138 levels, thereby linking Matrin-3 to dendritic spine morphogenesis regulation. Biochemical pull-down assays to identify Matrin-3 as pri/pre-miR-138-binding protein; Matrin-3 loss-of-function in HEK293 cells and primary neurons with miRNA quantification Neurobiology of learning and memory Medium 30790622
2018 MATR3 directly binds the lncRNA SNHG1 in neuroblastoma cells, validated by Western blot and RNA immunoprecipitation (RIP); this interaction is implicated in RNA splicing events that promote neuroblastoma progression. RNA-protein pull-down coupled with LC-MS/MS, followed by RIP validation and DeepBind motif analysis Journal of proteome research Medium 30516047
2020 The MATR3 S85C mutation causes loss of MATR3 protein in cell bodies of Purkinje cells and motor neurons in a knock-in mouse model, producing early-stage ALS features including motor impairment, muscle atrophy, neuromuscular junction defects, and neuroinflammation, suggesting loss of MATR3 function contributes to neurodegeneration. CRISPR/Cas9 S85C knock-in mouse model; behavioral testing, neuropathological analysis, immunohistochemistry Nature communications High 33082323
2020 Wild-type and mutant MATR3 expression in Drosophila motor neurons reduces climbing ability and lifespan; expression in indirect flight muscles causes muscle degeneration. Knockdown of axonal transport genes enhances MATR3 mutant toxicity and increases MATR3 protein insolubility, implicating axonal transport dysfunction in MATR3-linked disease pathogenesis. Transgenic Drosophila expressing human MATR3 variants; behavioral assays, candidate modifier screen, protein solubility analysis FEBS letters Medium 32515490
2020 MATR3 binds to sequences within intron-1 of the Charme lncRNA (pCharme) together with PTBP1 and other splicing regulators to form nuclear aggregates; targeted deletion of intron-1 releases pCharme from chromatin and causes cardiac defects, establishing that the MATR3/PTBP1 interaction with pCharme is required for chromatin domain formation during myogenesis. RNA antisense purification (RAP) coupled with mass spectrometry; CRISPR-Cas9 intron deletion in mouse; loss-of-function analyses Cell reports High 33357424
2021 MATR3 binds to the OCT4 and YTHDF1 promoters to favor their expression; additionally, MATR3 is recruited to ribosomes and controls translation of pluripotency transcripts (NANOG, LIN28A) by direct binding and stabilization. MATR3 downregulation disrupts pluripotency regulators OCT4, NANOG, and LIN28A through pleiotropic transcriptional, translational, and epitranscriptomic mechanisms in human iPSCs. MATR3 knockdown in hiPSCs; ChIP for promoter binding; ribosome fractionation; RNA immunoprecipitation; translation efficiency assays iScience Medium 33733063
2022 Matr3 suppresses oxLDL-induced macrophage inflammatory responses by inhibiting MAPK pro-inflammatory signaling through m6A-mediated mRNA decay; mechanistically, Matr3 regulates the formation of the Mettl3-Mettl14 methyltransferase complex. Matr3 expression decreases in oxLDL-stimulated macrophages and overexpression alleviates atherosclerosis in vivo. Co-immunoprecipitation; m6A sequencing; Matr3 overexpression in mouse atherosclerosis model; macrophage loss-of-function assays Clinical immunology (Orlando, Fla.) Medium 36368640
2023 MATR3 forms a meshwork with antisense LINE1 (AS L1) RNAs via phase separation, providing a dynamic platform for higher-order chromatin spatial organization. MATR3 and AS L1 RNAs regulate each other's nuclear localization. MATR3 depletion redistributes H3K27me3-modified chromatin and decreases intra-TAD interactions in TADs that transcribe MATR3-associated AS L1 RNAs. ALS-associated MATR3 mutants alter the biophysical properties of this meshwork. MATR3 depletion experiments; Hi-C for TAD analysis; ATAC-seq; ChIP for H3K27me3; phase-separation assays; fluorescence imaging EMBO reports High 37381832
2023 MATR3 acts as an endogenous inhibitor of DUX4 in FSHD; MATR3 physically binds to the DUX4 DNA-binding domain, blocking DUX4-mediated transcription and rescuing cell viability and myogenic differentiation of FSHD muscle cells. A shorter MATR3 fragment is necessary and sufficient to block DUX4 toxicity. Co-immunoprecipitation/pull-down; reporter and transcriptional assays; FSHD cell rescue experiments; domain-mapping with MATR3 fragments Cell reports High 37703175
2023 CTSB S-nitrosylation promotes dephosphorylation and nuclear translocation of ADD1, which recruits MATR3 and ADAR1 to CTSB mRNA, enabling ADAR1-mediated A-to-I RNA editing of CTSB mRNA. This editing allows HuR binding to CTSB mRNA, increasing its stability and expression in a feedforward loop. Co-immunoprecipitation; RNA immunoprecipitation; A-to-I editing assays; nuclear translocation imaging; loss-of-function experiments Cell research Medium 37156877
2023 MATR3 loss leads to cryptic exon inclusion in many transcripts (cryptic splicing repression function). The ALS-linked S85C variant reduces MATR3 solubility but does not impair RNA binding. A novel neurodevelopmental disease-associated M548T variant (in RRM2) reduces solubility, impairs RNA binding, and impairs cryptic splicing repression. RNA-seq after MATR3 knockdown/knockout; biochemical solubility assays; RNA-binding assays with mutant proteins FEBS letters Medium 38320753
2024 MATR3 binds DHX58 mRNA through its RRM domain and recruits YTHDF2 (an m6A reader), leading to degradation of DHX58 mRNA and suppression of the type I IFN signaling pathway in liver cancer cells. MATR3 knockout triggers innate immune responses and stimulates CD8+ T cells. RNA immunoprecipitation; RRM domain mutagenesis; YTHDF2 co-IP; RNA-seq; in vivo MATR3 knockout models Cancer letters Medium 39276912
2024 MATR3, FUS, and hnRNPA1 regulate UNC13A expression by binding to and downregulating REST mRNA, thereby promoting UNC13A transcription. REST overexpression occurs in motor neurons of familial and sporadic ALS patients upon loss of these RBPs. RNA-binding assays; knockdown in cultured cells; REST mRNA quantification; human ALS motor neuron analysis bioRxivpreprint Medium bio_10.1101_2024.10.22.619761
2025 IFN-I signaling phospho-regulates MATR3's RNA-binding activity; MATR3 is identified as an interferon-regulated RNA-binding protein (IR-RBP) whose association with RNA is modulated by IFN-I-driven phosphorylation. RNA interactome capture (RIC) before/after IFN-I treatment; phosphorylation state profiling by modified RIC; experimental validation of phospho-regulation bioRxivpreprint Medium bio_10.1101_2025.05.30.656608
2025 EZH2-mediated methylation of DDX1 at K234 disrupts DDX1's interaction with splicing factors, promoting exon 14 skipping in MATR3 (producing a truncated MATR3 isoform). This truncated MATR3 disrupts nuclear architecture, increases chromatin accessibility, and activates Wnt signaling, leading to nucleus pulposus cell senescence and apoptosis in intervertebral disc degeneration. In vitro and in vivo models; splicing analysis; chromatin accessibility (ATAC-seq); co-IP of DDX1 with splicing factors; mRNA delivery of full-length MATR3 via nanoparticles Nature communications Medium 40610464
2025 ASFV p30 protein interacts with host MATR3 and co-localizes with it in the cytoplasm; MATR3 promotes ASFV replication, and ASFV infection upregulates MATR3 expression. Mass spectrometry identification of p30 interactors; immunoprecipitation validation; co-localization imaging; MATR3 knockdown/overexpression with ASFV replication assay Veterinary microbiology Medium 39919500
2026 MATR3 interacts endogenously with METTL3 and stabilizes METTL3 protein by inhibiting its proteasomal degradation. METTL3 then mediates m6A modification of MSI2 mRNA, enhancing MSI2 stability and activating the Wnt/β-catenin pathway to promote atrial fibrosis. Co-immunoprecipitation; MeRIP-qPCR for m6A on MSI2 mRNA; MATR3 knockdown in human atrial fibroblasts; Ang-II mouse model with in vivo silencing Journal of bioenergetics and biomembranes Medium 41849072

Source papers

Stage 0 corpus · 73 papers · ranked by NIH iCite citations
Year Title Journal Citations PMID
2006 Global, in vivo, and site-specific phosphorylation dynamics in signaling networks. Cell 2861 17081983
2005 Towards a proteome-scale map of the human protein-protein interaction network. Nature 2090 16189514
2012 Insights into RNA biology from an atlas of mammalian mRNA-binding proteins. Cell 1718 22658674
2002 Generation and initial analysis of more than 15,000 full-length human and mouse cDNA sequences. Proceedings of the National Academy of Sciences of the United States of America 1479 12477932
2011 Systematic and quantitative assessment of the ubiquitin-modified proteome. Molecular cell 1334 21906983
2004 Large-scale characterization of HeLa cell nuclear phosphoproteins. Proceedings of the National Academy of Sciences of the United States of America 1159 15302935
2017 Architecture of the human interactome defines protein communities and disease networks. Nature 1085 28514442
2015 A human interactome in three quantitative dimensions organized by stoichiometries and abundances. Cell 1015 26496610
2014 A proteome-scale map of the human interactome network. Cell 977 25416956
2012 The mRNA-bound proteome and its global occupancy profile on protein-coding transcripts. Molecular cell 973 22681889
2020 A reference map of the human binary protein interactome. Nature 849 32296183
2018 VIRMA mediates preferential m6A mRNA methylation in 3'UTR and near stop codon and associates with alternative polyadenylation. Cell discovery 829 29507755
2003 Complete sequencing and characterization of 21,243 full-length human cDNAs. Nature genetics 754 14702039
2011 A proteome-wide, quantitative survey of in vivo ubiquitylation sites reveals widespread regulatory roles. Molecular & cellular proteomics : MCP 749 21890473
2007 Large-scale mapping of human protein-protein interactions by mass spectrometry. Molecular systems biology 733 17353931
2021 Dual proteome-scale networks reveal cell-specific remodeling of the human interactome. Cell 705 33961781
2012 A census of human soluble protein complexes. Cell 689 22939629
2011 Phylogenetic-based propagation of functional annotations within the Gene Ontology consortium. Briefings in bioinformatics 656 21873635
2006 A protein-protein interaction network for human inherited ataxias and disorders of Purkinje cell degeneration. Cell 610 16713569
2011 Global landscape of HIV-human protein complexes. Nature 593 22190034
2018 High-Density Proximity Mapping Reveals the Subcellular Organization of mRNA-Associated Granules and Bodies. Molecular cell 580 29395067
2017 Anticancer sulfonamides target splicing by inducing RBM39 degradation via recruitment to DCAF15. Science (New York, N.Y.) 533 28302793
2004 The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC). Genome research 438 15489334
2015 A Dynamic Protein Interaction Landscape of the Human Centrosome-Cilium Interface. Cell 433 26638075
2022 OpenCell: Endogenous tagging for the cartography of human cellular organization. Science (New York, N.Y.) 432 35271311
2010 Global analysis of TDP-43 interacting proteins reveals strong association with RNA splicing and translation machinery. Journal of proteome research 422 20020773
2010 Systematic analysis of human protein complexes identifies chromosome segregation proteins. Science (New York, N.Y.) 421 20360068
2015 Panorama of ancient metazoan macromolecular complexes. Nature 407 26344197
2011 IFIT1 is an antiviral protein that recognizes 5'-triphosphate RNA. Nature immunology 405 21642987
2001 The fate of dsRNA in the nucleus: a p54(nrb)-containing complex mediates the nuclear retention of promiscuously A-to-I edited RNAs. Cell 404 11525732
2015 MATR3 disruption in human and mouse associated with bicuspid aortic valve, aortic coarctation and patent ductus arteriosus. Human molecular genetics 84 25574029
2015 Replication study of MATR3 in familial and sporadic amyotrophic lateral sclerosis. Neurobiology of aging 57 26493020
2015 Mutational analysis of MATR3 in Taiwanese patients with amyotrophic lateral sclerosis. Neurobiology of aging 54 25771394
2022 Matr3 reshapes m6A modification complex to alleviate macrophage inflammation during atherosclerosis. Clinical immunology (Orlando, Fla.) 39 36368640
2020 Selective neuronal degeneration in MATR3 S85C knock-in mouse model of early-stage ALS. Nature communications 37 33082323
2018 RNA-Binding Proteomics Reveals MATR3 Interacting with lncRNA SNHG1 To Enhance Neuroblastoma Progression. Journal of proteome research 31 30516047
2020 Intronic Determinants Coordinate Charme lncRNA Nuclear Activity through the Interaction with MATR3 and PTBP1. Cell reports 30 33357424
2015 MATR3 mutation analysis in a Chinese cohort with sporadic amyotrophic lateral sclerosis. Neurobiology of aging 28 26708275
2018 Analysis of spinal and muscle pathology in transgenic mice overexpressing wild-type and ALS-linked mutant MATR3. Acta neuropathologica communications 22 30563574
2019 A mutant MATR3 mouse model to explain multisystem proteinopathy. The Journal of pathology 21 31056746
2021 Multilayer and MATR3-dependent regulation of mRNAs maintains pluripotency in human induced pluripotent stem cells. iScience 18 33733063
2023 MATR3-antisense LINE1 RNA meshwork scaffolds higher-order chromatin organization. EMBO reports 17 37381832
2014 pY RNA1-s2: a highly retina-enriched small RNA that selectively binds to Matrin 3 (Matr3). PloS one 16 24558381
2019 The nuclear matrix protein Matr3 regulates processing of the synaptic microRNA-138-5p. Neurobiology of learning and memory 15 30790622
2014 Mutation analysis of MATR3 in Australian familial amyotrophic lateral sclerosis. Neurobiology of aging 15 25523636
2023 Cathepsin B S-nitrosylation promotes ADAR1-mediated editing of its own mRNA transcript via an ADD1/MATR3 regulatory axis. Cell research 14 37156877
2015 Impairment of respiratory function in late-onset distal myopathy due to MATR3 Mutation. Muscle & nerve 12 25677933
2024 MATR3 pathogenic variants differentially impair its cryptic splicing repression function. FEBS letters 11 38320753
2011 A study of FHL1, BAG3, MATR3, PTRF and TCAP in Australian muscular dystrophy patients. Neuromuscular disorders : NMD 11 21683594
2022 Selective Loss of MATR3 in Spinal Interneurons, Upper Motor Neurons and Hippocampal CA1 Neurons in a MATR3 S85C Knock-In Mouse Model of Amyotrophic Lateral Sclerosis. Biology 9 35205163
2021 MATR3 F115C knock-in mice do not exhibit motor defects or neuropathological features of ALS. Biochemical and biophysical research communications 9 34182213
2020 Knockdown of genes involved in axonal transport enhances the toxicity of human neuromuscular disease-linked MATR3 mutations in Drosophila. FEBS letters 9 32515490
2020 The Impact of ALS-Associated Genes hnRNPA1, MATR3, VCP and UBQLN2 on the Severity of TDP-43 Aggregation. Cells 8 32731393
2025 DDX1 methylation mediated MATR3 splicing regulates intervertebral disc degeneration by initiating chromatin reprogramming. Nature communications 7 40610464
2024 MATR3 promotes liver cancer progression by suppressing DHX58-mediated type I interferon response. Cancer letters 7 39276912
2023 MATR3 is an endogenous inhibitor of DUX4 in FSHD muscular dystrophy. Cell reports 7 37703175
2024 MATR3's Role beyond the Nuclear Matrix: From Gene Regulation to Its Implications in Amyotrophic Lateral Sclerosis and Other Diseases. Cells 6 38891112
2021 First Family of MATR3-Related Distal Myopathy From Italy: The Role of Muscle Biopsy in the Diagnosis and Characterization of a Still Poorly Understood Disease. Frontiers in neurology 6 34659085
2022 Matrin3 (MATR3) Expression Is Associated with Hemophagocytosis. Biomedicines 4 36140262
2006 Myotilin is not the causative gene for vocal cord and pharyngeal weakness with distal myopathy (VCPDM). Annals of human genetics 4 16674563
2023 MATR3 P154S knock-in mice do not exhibit motor, muscle or neuropathologic features of ALS. Biochemical and biophysical research communications 3 36689813
2023 LncRNA15691 promotes T-ALL infiltration by upregulating CCR9 via increased MATR3 stability. Journal of leukocyte biology 3 36822174
2023 The effect on m6A methylation writer complex by the reduced MATR3 in pterygium. Acta histochemica 3 37913560
2022 Expanding the Phenotypic Spectrum of Vocal Cord and Pharyngeal Weakness With Distal Myopathy due to the p.S85C MATR3 Mutation. Neurology. Genetics 2 35812165
2018 Generation and characterization of a human iPSC line from an ALS patient carrying the Q66K-MATR3 mutation. Stem cell research 2 30366341
2025 ASFV p30 interacts with CCAR2 and MATR3 to promote ASFV replication. Veterinary microbiology 1 39919500
2025 Sex-dependent epigenetic disruption of YY1 binding by prenatal BPA exposure downregulates Matr3 and alters Agap1 splicing in the offspring hippocampus. Biology of sex differences 1 40790242
2024 Knockout of Dectin-1 does not modify disease onset or progression in a MATR3 S85C knock-in mouse model of ALS. Heliyon 1 39323783
2020 Generation of two induced pluripotent stem cell (iPSC) lines from an ALS patient with simultaneous mutations in KIF5A and MATR3 genes. Stem cell research 1 33388707
2026 MATR3 regulates METTL3-Mediated m6A modification of MSI2 mRNA to activate the Wnt/β-Catenin pathway, exacerbating myocardial fibrosis and atrial fibrillation. Journal of bioenergetics and biomembranes 0 41849072
2025 Autosomal dominant distal myopathy due to p.Ser85Cys mutation in the MATR3 gene: Novel case series and literature review. Revue neurologique 0 40447473
2024 Asymmetric scapuloperoneal phenotype of MATR3-related distal myopathy: case series. Frontiers in genetics 0 39192891
2014 Obstetric risk in patients with myopathy due to MATR3 mutations. Molecular genetics and metabolism reports 0 28649522