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ZFC3H1

Zinc finger C3H1 domain-containing protein · UniProt O60293

Length
1989 aa
Mass
226.4 kDa
Annotated
2026-06-11
23 papers in source corpus 17 papers cited in narrative 17 extracted findings
Cross-family judge vs UniProt: tie faithfulness: 7/9 claims corpus-supported (78%)

Mechanistic narrative

Synthesis pass · prose summary of the discoveries below

ZFC3H1 is a central scaffold of the poly(A) tail exosome targeting (PAXT) connection, a nuclear RNA surveillance system that routes polyadenylated transcripts toward exosomal degradation (PMID:27871484). It forms a core dimer with the RNA helicase MTR4 and bridges MTR4 to the nuclear poly(A)-binding protein PABPN1; this PAXT engagement of MTR4 is mutually exclusive with the NEXT adaptor module (ZCCHC8/RBM7), so that NEXT and PAXT compete for MTR4 to target transcripts of differing maturation status (PMID:27871484). The MTR4-ZFC3H1 dimer additionally recruits transient subunits ZC3H3, RBM26 and RBM27, and loss of any component stabilizes PAXT substrates (PMID:31950173). Mechanistically, ZFC3H1 is loaded co-transcriptionally onto the first exon/intron of RNA precursors in a 'closed' conformation that blocks exosome recruitment; short, few-exon RNAs recruit ZC3H3 and RBM26/27 to the 3' end to trigger ZFC3H1 'opening' and degradation, whereas longer transcripts are diverted to nuclear export, establishing a decoupled loading-and-activation switch that pre-configures RNA fate (PMID:39461342). The MTR4-ZFC3H1 interaction is negatively regulated by NRDE2, which binds MTR4 via a conserved MTR4-interacting domain and competes with ZFC3H1 for key MTR4 residues to lock the helicase closed (PMID:30842217), and a distinct PAXT branch targeting short adenylated RNAs is engaged through a direct ARS2-ZFC3H1 interaction that competes with ZC3H18 (PMID:37889751). Beyond degradation, ZFC3H1 acts as a nuclear retention factor: when exosome function is lost it forms nuclear condensates that retain polyadenylated RNAs away from nuclear speckles and the AlyREF export machinery, with condensation and degradation served by separable domains (PMID:29768216, PMID:34530450). It enforces nuclear retention of intronic polyadenylated transcripts bearing intact 5' splice site motifs in the same pathway as U1-70K, sequestering them into nuclear speckles (PMID:35351812), and engages the m6A readers YTHDC1/YTHDC2 to retain 5'SS-containing mRNAs (PMID:39626965). PAXT is recruited by ZFC3H1 to transcription start sites genome-wide, where its loss abolishes recruitment of PAP-gamma and other subunits and increases PROMPT abundance (PMID:37875486). Through this surveillance activity ZFC3H1 also supports embryonic stem cell differentiation, preventing nonspecific RNA from compromising PRC2 stability and chromatin occupancy (PMID:31722198), and restrains aberrant cytoplasmic accumulation and translation of unprocessed lncRNAs (PMID:28733371).

Mechanistic history

Synthesis pass · year-by-year structured walk · 12 steps
  1. 2016 High

    Established ZFC3H1 as the adaptor defining a distinct MTR4-dependent surveillance pathway, answering how polyadenylated transcripts are selectively delivered to the nuclear exosome.

    Evidence Reciprocal Co-IP and depletion with RNA-seq in human cells, defining PAXT versus NEXT mutual exclusivity on MTR4

    PMID:27871484

    Open questions at the time
    • Did not resolve the structural basis of the MTR4-ZFC3H1 contact
    • Substrate selectivity rules between NEXT and PAXT not defined
  2. 2017 High

    Showed the MTR4/ZFC3H1 complex prevents cytoplasmic escape of prematurely terminated and antisense RNAs, linking failed nuclear surveillance to translational disruption.

    Evidence Complex purification, siRNA knockdown, fractionation, polysome profiling and RIP in human cells

    PMID:28733371

    Open questions at the time
    • Mechanism distinguishing retention from degradation not separated
    • Did not define how transcripts escape to the cytoplasm
  3. 2018 High

    Defined ZFC3H1 as a nuclear pA+ RNA retention factor that counteracts AlyREF-mediated export, separating retention from degradation as a function.

    Evidence RNA-FISH/IF co-localization, knockdown, fractionation and AlyREF epistasis in human cells

    PMID:29768216

    Open questions at the time
    • Physical basis of foci formation not yet determined
    • Selectivity of which transcripts are retained unresolved
  4. 2019 High

    Resolved a negative regulatory switch: NRDE2 competes with ZFC3H1 for MTR4 to lock the helicase closed and block exosome recruitment.

    Evidence Structural and biochemical interaction assays with MTR4-interacting-domain mutagenesis and Co-IP

    PMID:30842217

    Open questions at the time
    • Cellular conditions governing NRDE2-versus-ZFC3H1 occupancy of MTR4 not defined
  5. 2019 High

    Connected ZFC3H1 surveillance to developmental gene control, showing its loss destabilizes PRC2 via nonspecific RNA and impairs ES cell differentiation.

    Evidence CRISPR knockout in mouse ES cells with H3K27me3/PRC2 ChIP-seq, RNA-seq and RNA-IP

    PMID:31722198

    Open questions at the time
    • Direct versus indirect effect on PRC2 not fully separated
    • Which RNAs accumulate on PRC2 not identified
  6. 2020 High

    Expanded the PAXT subunit roster, identifying ZC3H3/RBM26/RBM27 as components recruited to the MTR4-ZFC3H1 dimer required for substrate turnover.

    Evidence Nuclear pA+-RNA proteomics, Co-IP of MTR4-ZFC3H1, and knockdown RNA-seq in human cells

    PMID:31950173

    Open questions at the time
    • Stoichiometry and assembly order of transient subunits unresolved
    • Whether subunits are constitutive or substrate-induced not settled at the time
  7. 2021 Medium

    Dissected ZFC3H1 into separable domains for condensation versus degradation, showing condensate formation drives nuclear RNA retention independent of degradation.

    Evidence Live-cell imaging, domain deletion/mutation, RNA-FISH and speckle microscopy under exosome inactivation

    PMID:34530450

    Open questions at the time
    • Biophysical determinants of condensation not characterized
    • Single lab; physiological trigger for condensation in vivo unclear
  8. 2022 High

    Showed ZFC3H1 enforces nuclear retention of intronic polyadenylated transcripts with intact 5'SS motifs, placing it in a U1-70K-coupled surveillance pathway.

    Evidence Fractionation-seq, reporter mRNA assays, speckle disruption and ZFC3H1/U1-70K epistasis

    PMID:35351812

    Open questions at the time
    • Direct ZFC3H1-U1 snRNP contact not demonstrated
    • How 5'SS recognition feeds into retention not molecularly defined
  9. 2023 Medium

    Defined an ARS2-ZFC3H1 interaction branch for short adenylated RNAs, explaining how ZC3H18 partitions activity between NEXT and PAXT.

    Evidence Mutagenesis of acidic short linear motifs, competitive binding assays and KD/KO RNA-seq

    PMID:37889751

    Open questions at the time
    • Structural detail of the ARS2 epitope contact not resolved
    • In vivo balance of ZC3H18 versus ZFC3H1 for ARS2 not quantified
  10. 2023 High

    Mapped ZFC3H1-dependent PAXT recruitment to transcription start sites genome-wide, linking PAXT to PROMPT polyadenylation and turnover.

    Evidence ChIP-seq of ZFC3H1/RBM27/PAP-gamma, ZFC3H1 KO RNA-seq and proteomics

    PMID:37875486

    Open questions at the time
    • How ZFC3H1 is targeted to specific TSSs unresolved
    • Role of PAP-gamma polyadenylation step mechanistically incomplete
  11. 2024 High

    Resolved a decoupled loading-and-activation model: co-transcriptional loading in a closed state followed by 3'-end-triggered opening that pre-configures degradation versus export.

    Evidence iCLIP/eCLIP, domain mutants, Co-IP, fractionation and nascent RNA analysis

    PMID:39461342

    Open questions at the time
    • Exact conformational change accompanying opening not structurally defined
    • Counting mechanism for exon number not elucidated
  12. 2024 Medium

    Connected ZFC3H1 retention to m6A reading, showing YTHDC1/2 partner with ZFC3H1 and U1-70K to retain 5'SS-containing mRNAs.

    Evidence Co-IP, knockdown, reporter assays, RNA-FISH and m6A inhibition in human cells

    PMID:39626965

    Open questions at the time
    • Direct versus indirect ZFC3H1-YTHDC1/2 contact not separated
    • Single study; physiological scope of m6A-dependent retention unclear

Open questions

Synthesis pass · forward-looking unresolved questions
  • How ZFC3H1 substrate-specificity, condensation, and chromatin-proximal recruitment are integrated into a single regulatory logic that decides RNA fate remains unresolved.
  • No high-resolution structure of the assembled PAXT-ZFC3H1 complex
  • Determinants distinguishing degraded versus exported transcripts only partially defined
  • Reported roles in HIV-1 latency, PRC2/differentiation, and disease-associated mRNAs not unified mechanistically

Mechanism profile

Synthesis pass · controlled-vocabulary classification · explore literature graph →
Molecular activity
GO:0003723 RNA binding 3 GO:0060090 molecular adaptor activity 2 GO:0098772 molecular function regulator activity 2
Localization
GO:0005634 nucleus 3 GO:0005654 nucleoplasm 3
Pathway
R-HSA-8953854 Metabolism of RNA 4 R-HSA-9609507 Protein localization 3 R-HSA-74160 Gene expression (Transcription) 1
Complex memberships
PAXT connection

Evidence

Reading pass · 17 per-paper findings extracted from the source corpus
Year Finding Method Journal Conf PMIDs
2016 ZFC3H1 is a central component of the poly(A) tail exosome targeting (PAXT) connection, acting as a link between the RNA helicase hMTR4 and the nuclear poly(A)-binding protein PABPN1 to facilitate nuclear exosome degradation of polyadenylated transcripts. ZFC3H1/PABPN1 and ZCCHC8/RBM7 contact hMTR4 in a mutually exclusive manner, establishing that NEXT and PAXT are distinct, competing hMTR4-adaptor complexes targeting transcripts of different maturation status. Protein co-immunoprecipitation, depletion/knockdown with RNA-seq readout, identification of mutual exclusivity by interaction assays Molecular cell High 27871484
2017 ZFC3H1 forms a distinct complex with MTR4 (separate from NEXT) that is required for nuclear surveillance of prematurely terminated RNAs (ptRNAs) and upstream antisense RNAs (uaRNAs). Knockdown of either Mtr4 or ZFC3H1 causes these lncRNAs to accumulate in the cytoplasm and associate with active ribosomes, leading to global repression of translation, establishing a role for the Mtr4/ZFC3H1 complex in preventing cytoplasmic transport and translational disruption. Complex purification/isolation, siRNA knockdown, cellular fractionation, polysome profiling, RNA immunoprecipitation Genes & development High 28733371
2018 ZFC3H1 is required for the formation of distinct nuclear foci containing polyadenylated RNA when exosome function is abolished. In the absence of ZFC3H1, selected polyadenylated RNAs (coding and non-coding) are exported to the cytoplasm via the mRNA export factor AlyREF, establishing ZFC3H1 as a central nuclear pA+ RNA retention factor that counteracts nuclear export activity. Co-localization imaging (RNA FISH + immunofluorescence), siRNA knockdown, cellular fractionation, AlyREF epistasis experiments Cell reports High 29768216
2018 ZFC3H1 physically associates with the HIV-1 TAR region and represses HIV-1 transcriptional output and RNAPII recruitment to the LTR. Knockdown of ZFC3H1 increases HIV-1 expression and reactivates HIV-1 from latently infected PBMCs. ChIP (chromatin immunoprecipitation), siRNA knockdown, reporter assays, flow cytometry of GFP-positive J-Lat cells, ex vivo PBMC infection PLoS pathogens Medium 29554134
2019 NRDE2 inhibits ZFC3H1 interaction with MTR4 by binding MTR4 via a conserved MTR4-interacting domain (MID), locking MTR4 in a closed conformation and thereby blocking exosome recruitment. Structural and biochemical data confirm that NRDE2 competes with ZFC3H1 for binding to key residues on MTR4. Structural analysis, biochemical interaction assays, mutagenesis of MTR4-interacting domain, Co-IP Genes & development High 30842217
2019 Knockout of ZFC3H1 in mouse embryonic stem cells impairs differentiation and leads to de-repression of PRC2-controlled developmental genes, paralleled by decreased PRC2 binding to chromatin, reduced H3K27 methylation, and compromised PRC2 complex stability due to elevated nonspecific RNA bound to PRC2 components. CRISPR/Cas9 knockout, ChIP-seq for H3K27me3 and PRC2, RNA-seq, RNA immunoprecipitation Cell reports High 31722198
2019 Celastramycin binds ZFC3H1 as a direct binding partner (pulled down by celastramycin), and ZFC3H1 mediates celastramycin's effects on HIF-1α and NF-κB protein levels, reactive oxygen species, and mitochondrial metabolism in pulmonary artery smooth muscle cells. Affinity pull-down with celastramycin, siRNA knockdown, western blotting, functional cellular assays Circulation research Medium 31195886
2020 ZFC3H1 functions as the core dimer partner of MTR4 in the PAXT connection, and three additional proteins—ZC3H3, RBM26, and RBM27—are required for PAXT function. ZC3H3 interacts directly with the MTR4-ZFC3H1 dimer, and loss of any newly identified component results in accumulation of PAXT substrates. Proteomics of nuclear pA+-RNA bound proteins, Co-IP of MTR4-ZFC3H1 complexes, siRNA knockdown with RNA-seq readout Nucleic acids research High 31950173
2021 Upon exosome inactivation, ZFC3H1 forms nuclear condensates that prevent polyadenylated RNA trafficking to nuclear speckles, thereby blocking export competence. Systematic domain mapping revealed that ZFC3H1 uses distinct domains for condensation and for RNA degradation; condensation activity is required for nuclear RNA retention but not for RNA degradation. Live-cell imaging, domain deletion/mutation analysis, RNA FISH, siRNA knockdown, fluorescence microscopy of nuclear speckles Nucleic acids research Medium 34530450
2022 ZFC3H1 is required for the nuclear retention and degradation of intronic polyadenylated (IPA) transcripts that contain intact 5' splice site (5'SS) motifs. ZFC3H1 sequesters mRNAs with 5'SS motifs into nuclear speckles to prevent their nuclear export, functioning in the same pathway as U1-70K (a component of the U1 snRNP). High-throughput sequencing of cellular fractions, reporter mRNA assays, siRNA knockdown, nuclear speckle disruption experiments, epistasis between ZFC3H1 and U1-70K RNA (New York, N.Y.) High 35351812
2023 Mutational analysis of ZFC3H1 uncovered a direct ARS2-ZFC3H1 interaction via acidic-rich short linear motifs that compete with ZC3H18 for a common ARS2 epitope. This reveals a separate PAXT branch targeting short adenylated RNAs and explains how ZC3H18 simultaneously promotes NEXT while antagonizing PAXT activity. Site-directed mutagenesis, Co-IP, competitive binding assays, RNA-seq after KD/KO Cell reports Medium 37889751
2023 ZFC3H1 is required for PAXT recruitment to transcription start sites (TSSs) of hundreds of genes; loss of ZFC3H1 abolishes recruitment of all PAXT subunits including PAPγ to TSSs and concomitantly increases the abundance of PROMPTs at those sites. ZFC3H1, MTR4, and PAPγ are all implicated in polyadenylation of PROMPTs. ChIP-seq / genome-wide binding mapping of ZFC3H1, RBM27, and PAPγ; ZFC3H1 KO with RNA-seq; proteomics Nature communications High 37875486
2024 ZFC3H1 is co-transcriptionally loaded onto the first exon/intron of RNA precursors in a 'closed' conformation that blocks exosome recruitment. Upon RNA processing, short RNAs with fewer exons recruit transient PAXT components ZC3H3 and RBM26/27 to the 3' end, triggering ZFC3H1 'opening' and subsequent exosomal degradation, whereas longer RNAs with more exons are directed to nuclear export. This establishes a decoupled loading-and-activation mechanism for ZFC3H1 that pre-configures RNA fate. iCLIP/eCLIP mapping, domain mutant analysis, Co-IP, RNA-seq fractionation, nascent RNA analysis Molecular cell High 39461342
2024 YTHDC1 and YTHDC2 (YTH-domain m6A reader proteins) interact with ZFC3H1 and U1-70K, and are required for nuclear retention of mRNAs with intact 5'SS motifs. Disruption of m6A deposition inhibits nuclear retention of these transcripts and their accumulation in YTHDC1-enriched foci adjacent to nuclear speckles. Co-IP (ZFC3H1 with YTHDC1/2), siRNA knockdown, reporter mRNA assays, RNA FISH, m6A inhibition experiments Life science alliance Medium 39626965
2014 ZFC3H1 (also known as CCDC131/CSRC2) was identified as a direct binding protein of Celastramycin A by affinity pull-down screening. Knockdown of ZFC3H1 reduced TNFα-induced IL-8 expression, and reporter assays showed ZFC3H1 participates in transcriptional activation of IL-8. UV-irradiation experiments suggested ZFC3H1 may indirectly interact with ERCC1 in an activated DNA repair complex. Celastramycin A affinity pull-down screen, siRNA knockdown, IL-8 reporter assay, Co-IP suggestion PloS one Medium 25268596
2022 ZFC3H1 participates in human telomerase RNA (hTR) biogenesis via pathways related to the polyadenylated RNA degradation mechanism, as determined by knockdown experiments examining hTR isoforms and localization. siRNA knockdown, RT-PCR analysis of hTR isoforms, cellular fractionation Biomedicines Low 35740297
2024 ZFC3H1 protein competes with lncRNA MSL3P1 for binding to CUL3 mRNA; MSL3P1 prevents ZFC3H1-mediated degradation and cytoplasmic export blockade of CUL3 mRNA by competitive binding, demonstrating ZFC3H1's role in targeting specific mRNAs for exosomal degradation and nuclear retention. RNA immunoprecipitation, siRNA knockdown of ZFC3H1, RNA pull-down competition assays, cellular fractionation Molecular cancer research : MCR Low 38718076

Source papers

Stage 0 corpus · 23 papers · ranked by NIH iCite citations
Year Title Journal Citations PMID
2016 Identification of a Nuclear Exosome Decay Pathway for Processed Transcripts. Molecular cell 230 27871484
2017 An Mtr4/ZFC3H1 complex facilitates turnover of unstable nuclear RNAs to prevent their cytoplasmic transport and global translational repression. Genes & development 113 28733371
2018 The RNA Exosome Adaptor ZFC3H1 Functionally Competes with Nuclear Export Activity to Retain Target Transcripts. Cell reports 75 29768216
1999 cSrc is a major cytosolic tyrosine kinase in vascular tissue. Canadian journal of physiology and pharmacology 71 10543724
2020 The human ZC3H3 and RBM26/27 proteins are critical for PAXT-mediated nuclear RNA decay. Nucleic acids research 62 31950173
2019 Identification of Celastramycin as a Novel Therapeutic Agent for Pulmonary Arterial Hypertension. Circulation research 46 31195886
2007 A gene signature of 8 genes could identify the risk of recurrence and progression in Dukes' B colon cancer patients. Oncology reports 45 17390049
2019 NRDE2 negatively regulates exosome functions by inhibiting MTR4 recruitment and exosome interaction. Genes & development 44 30842217
2019 A Functional Link between Nuclear RNA Decay and Transcriptional Control Mediated by the Polycomb Repressive Complex 2. Cell reports 34 31722198
2022 ZFC3H1 and U1-70K promote the nuclear retention of mRNAs with 5' splice site motifs within nuclear speckles. RNA (New York, N.Y.) 23 35351812
2021 ZFC3H1 prevents RNA trafficking into nuclear speckles through condensation. Nucleic acids research 20 34530450
2018 Nuclear RNA surveillance complexes silence HIV-1 transcription. PLoS pathogens 20 29554134
2023 Dual agonistic and antagonistic roles of ZC3H18 provide for co-activation of distinct nuclear RNA decay pathways. Cell reports 14 37889751
2014 ZFC3H1, a zinc finger protein, modulates IL-8 transcription by binding with celastramycin A, a potential immune suppressor. PloS one 13 25268596
2023 PAPγ associates with PAXT nuclear exosome to control the abundance of PROMPT ncRNAs. Nature communications 12 37875486
2020 Use of peripheral blood transcriptomic biomarkers to distinguish high-grade cervical squamous intraepithelial lesions from low-grade lesions. Oncology letters 12 32765790
2022 Construction of AP003469.4-miRNAs-mRNAs ceRNA network to reveal potential biomarkers for hepatocellular carcinoma. American journal of cancer research 10 35530270
2024 N-6-methyladenosine (m6A) promotes the nuclear retention of mRNAs with intact 5' splice site motifs. Life science alliance 8 39626965
2024 Dual modes of ZFC3H1 confer selectivity in nuclear RNA sorting. Molecular cell 6 39461342
2022 Role of RNA Biogenesis Factors in the Processing and Transport of Human Telomerase RNA. Biomedicines 5 35740297
2024 Long Noncoding RNA MSL3P1 Regulates CUL3 mRNA Cytoplasmic Transport and Stability and Promotes Lung Adenocarcinoma Metastasis. Molecular cancer research : MCR 3 38718076
2024 Genome-wide association study for conformation traits in Ayrshire cattle. Animal science journal = Nihon chikusan Gakkaiho 1 39165009
2026 Molecular mechanisms linking cadmium chloride exposure to ankylosing spondylitis: an integrative network-based study. Naunyn-Schmiedeberg's archives of pharmacology 0 42113191

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