Affinage

NOP56

Nucleolar protein 56 · UniProt O00567

Round 2 corrected
Length
594 aa
Mass
66.0 kDa
Annotated
2026-04-29
67 papers in source corpus 13 papers cited in narrative 14 extracted findings

Mechanistic narrative

Synthesis pass · prose summary of the discoveries below

NOP56 is a conserved core subunit of box C/D small nucleolar ribonucleoprotein (snoRNP) complexes essential for 2'-O-methylation of ribosomal RNA and ribosome biogenesis. Its N-terminal domain forms an exceptionally stable interaction with fibrillarin (the methyltransferase catalytic subunit), recruiting it to the guide RNA, while its coiled-coil domain is dispensable for snoRNP assembly but required for proper RNP architecture and guided nucleotide methylation; NOP56 also directly contacts the target RNA substrate, contributing to catalysis beyond a purely scaffolding role (PMID:16601205, PMID:22496443, PMID:33483369). NOP56 protein levels are autoregulated through an intronic snoRD86-based RNA structure feedback mechanism that couples alternative splicing to nonsense-mediated decay of NOP56 mRNA, and a reciprocal positive feedback loop with MYC links NOP56 to ribosome biogenesis-driven cell transformation and cancer progression (PMID:30220559, PMID:24013231, PMID:41827688). Loss of NOP56 in zebrafish causes severe cerebellar agenesis and neurodegeneration, and a GGCCTG intronic repeat expansion in NOP56 undergoes repeat-associated non-AUG (RAN) translation in all sense-strand reading frames, connecting NOP56 to neurodegenerative disease mechanisms (PMID:36009362, PMID:40015643).

Mechanistic history

Synthesis pass · year-by-year structured walk · 11 steps
  1. 2006 High

    Determining whether the coiled-coil domain of Nop56/58 functions in snoRNP assembly or catalysis resolved its role: the domain is dispensable for RNP formation but essential for guided 2'-O-methylation and proper RNP architecture, and Nop56/58 self-dimerization and fibrillarin binding are mutually exclusive.

    Evidence In vitro methylation assays with deletion/mutation constructs and nuclease probing of archaeal sRNPs

    PMID:16601205

    Open questions at the time
    • Whether the coiled-coil requirement is conserved in eukaryotic NOP56
    • Mechanism by which coiled-coil domain organizes dual catalytic sites
  2. 2007 High

    Solving the crystal structure of the archaeal Nop56/58–fibrillarin–SAM complex revealed a bipartite dimeric arrangement with intrinsic conformational flexibility, explaining how two catalytic sites can be positioned on a single bipartite C/D guide RNA.

    Evidence X-ray crystallography (2.7 Å) of Pyrococcus furiosus complex plus computational normal mode analysis

    PMID:17617422

    Open questions at the time
    • No eukaryotic structure available at the time
    • Structural basis of substrate RNA engagement unresolved
  3. 2009 High

    In vivo interaction mapping established that fibrillarin and Nop56 form a pre-assembled heterodimer before incorporation into box C/D snoRNPs, with the fibrillarin α-helix domain but not its GAR or RNA-binding domains mediating the interaction.

    Evidence In vivo relocalization/affinity-tag approach with co-immunoprecipitation and fluorescence microscopy in mammalian cells

    PMID:19331828

    Open questions at the time
    • Whether pre-assembly is obligatory or rate-limiting for snoRNP biogenesis
  4. 2012 High

    High-resolution structural and functional analysis of the Nop56/58 N-terminal domain demonstrated that it mediates an exceptionally stable fibrillarin interaction and directly contacts the target RNA substrate, establishing a catalytic contribution beyond scaffolding.

    Evidence X-ray crystallography (1.7 Å), site-directed mutagenesis, chemical/thermal denaturation, RNA cross-linking, and in vitro methylation assay on archaeal sRNPs

    PMID:22496443

    Open questions at the time
    • Precise residues contacting the substrate RNA not mapped
    • Whether NTD mutations affect rRNA methylation fidelity in vivo
  5. 2013 Medium

    Identifying NOP56 as a rate-limiting target hyperactivated by oncogenic Myc mutants linked snoRNP-mediated rRNA methylation to Myc-driven cell transformation, answering whether ribosome biogenesis components are functionally required for Myc oncogenesis.

    Evidence Gene expression profiling, siRNA knockdown and overexpression with cell transformation and in vivo tumor growth assays

    PMID:24013231

    Open questions at the time
    • Which specific rRNA methylation sites are rate-limiting for transformation
    • Whether NOP56 contributes to transformation independently of its snoRNP role
  6. 2018 High

    Discovery of a cis-acting autoregulatory feedback loop in which the intron-hosted snoRD86 RNA senses snoRNP core protein levels and switches NOP56 pre-mRNA splicing toward an NMD-destined isoform established a new paradigm for how snoRNP homeostasis is maintained.

    Evidence RNA splicing analysis, NMD pathway assays, RNP structure analysis, and alternative splicing mapping in human cells

    PMID:30220559

    Open questions at the time
    • Whether analogous mechanisms regulate NOP58 or other snoRNP genes
    • Quantitative contribution of the NMD pathway versus splicing regulation
  7. 2021 High

    Solving the eukaryotic Nop56 NTD–fibrillarin crystal structure revealed that the methyltransferase recruitment interface has diverged substantially from archaea, answering whether the archaeal structural paradigm generalizes to eukaryotes.

    Evidence High-resolution X-ray crystallography of yeast Nop1–Nop56 NTD complex with structural comparison to archaeal orthologs

    PMID:33483369

    Open questions at the time
    • No full-length eukaryotic box C/D snoRNP structure with guide RNA
    • How divergent interface relates to differences in eukaryotic snoRNP regulation
  8. 2022 Medium

    Zebrafish nop56 loss-of-function demonstrated that NOP56 is essential for vertebrate CNS development, with mutants exhibiting cerebellar agenesis, spinal neuron loss, and high apoptosis, connecting snoRNP function to neuronal survival.

    Evidence Zebrafish genetic knockout with fluorescence microscopy, apoptosis assays, gene expression analysis, and behavioral assays

    PMID:36009362

    Open questions at the time
    • Whether neurodegeneration results from global loss of rRNA methylation or specific snoRNA targets
    • Whether phenotype is cell-autonomous in neurons
  9. 2022 Medium

    Demonstrating synthetic lethality between NOP56 depletion and mTOR inhibition in KRAS-mutant lung cancer revealed that NOP56 regulates ROS homeostasis and that cells compensate for NOP56 loss through IRE1α–p38–mTOR signaling.

    Evidence siRNA/shRNA, CRISPR knockout, ROS measurement, Western blot, and xenograft tumor model in KRAS-mutant lung cancer cells

    PMID:35039048

    Open questions at the time
    • Whether ROS elevation is a direct consequence of impaired rRNA methylation or a secondary effect
    • Generalizability beyond KRAS-mutant contexts
  10. 2025 Medium

    Characterization of RAN translation from NOP56 GGCCTG intronic repeats showed cap-dependent initiation from near-cognate codons in all sense reading frames with repeat length–dependent enhancement, establishing a molecular mechanism for toxic protein production in NOP56 repeat expansion disease.

    Evidence Cell-free in vitro translation systems with repeat constructs of varying lengths and frameshifting analysis

    PMID:40015643

    Open questions at the time
    • Whether RAN translation products accumulate in patient neurons
    • Relative contribution of RAN products versus RNA toxicity to neurodegeneration
    • No in vivo validation yet
  11. 2026 Medium

    Identification of a NOP56–FBL–PI3K/AKT/CREB signaling axis in hepatocellular carcinoma and a reciprocal NOP56–MYC positive feedback loop in NSCLC extended NOP56's oncogenic roles beyond rRNA methylation to IRES-dependent translation regulation and growth signaling.

    Evidence Co-IP, rescue experiments, ChIP, bisulfite sequencing, luciferase reporters, RNA-seq, and xenograft models in HCC and NSCLC cells

    PMID:41568368 PMID:41827688

    Open questions at the time
    • Whether the PI3K/AKT activation is a direct consequence of altered ribosome function or a fibrillarin-specific effect
    • Whether the NOP56–MYC feedback loop operates in non-transformed cells
    • No structural basis for NOP56's role in IRES-dependent translation

Open questions

Synthesis pass · forward-looking unresolved questions
  • A complete high-resolution structure of a eukaryotic box C/D snoRNP with NOP56, NOP58, fibrillarin, 15.5K, and guide RNA bound to substrate rRNA is still lacking, and the precise mechanism by which NOP56 contributes to catalysis versus NOP58 within the asymmetric eukaryotic complex remains unresolved.
  • No full eukaryotic holo-snoRNP structure with substrate
  • Functional asymmetry between NOP56 and NOP58 not resolved
  • In vivo identification of NOP56-dependent rRNA methylation sites critical for disease phenotypes

Mechanism profile

Synthesis pass · controlled-vocabulary classification · explore literature graph →
Molecular activity
GO:0005198 structural molecule activity 4 GO:0003723 RNA binding 3
Localization
GO:0005730 nucleolus 3
Pathway
R-HSA-1643685 Disease 4 R-HSA-8953854 Metabolism of RNA 4 GO:0005730 nucleolus 3 R-HSA-392499 Metabolism of proteins 3
Partners
FBLMYCNHP2L1NOP58
Complex memberships
box C/D snoRNP

Evidence

Reading pass · 14 per-paper findings extracted from the source corpus
Year Finding Method Journal Conf PMIDs
2006 The coiled-coil domain of archaeal Nop56/58 is dispensable for sRNP assembly but critical for box C/D sRNP-guided nucleotide methylation. Nop56/58 self-dimerization and Nop56/58-fibrillarin dimerization are mutually exclusive protein-protein interactions. Deletion of the coiled-coil domain disrupts guided methylation from both box C/D and C'/D' RNP complexes and alters RNP structure, despite allowing functional complex assembly. Protein pull-down, site-directed mutagenesis, deletion constructs, in vitro methylation assay, nuclease probing of sRNP structure RNA (New York, N.Y.) High 16601205
2007 Crystal structure of the archaeal Nop56/58-fibrillarin complex from Pyrococcus furiosus bound with S-adenosyl-L-methionine reveals a bipartite dimeric arrangement that is a general feature across species. The conformation of Nop56/58 in this structure differs substantially from the earlier Archaeoglobus fulgidus structure, indicating intrinsic conformational flexibility. Computational normal mode analysis supports hinge motion within Nop56/58, suggesting that such flexibility allows simultaneous positioning of two catalytic sites at two target sites of a bipartite box C/D guide RNA. X-ray crystallography (2.7 Å), computational normal mode analysis Journal of molecular biology High 17617422
2009 Fibrillarin and Nop56 directly interact in vivo before being co-assembled into box C/D snoRNPs. This interaction does not require the glycine- and arginine-rich domain or the RNA-binding domain of fibrillarin, but depends on the alpha-helix domain of fibrillarin. No RNA is required to maintain the fibrillarin-Nop56 interaction. Altering the localization and mobility of core box C/D proteins (including Nop56) impairs their association with box C/D snoRNPs. In vivo relocalization/affinity-tag approach (B23 tag), co-immunoprecipitation, fluorescence microscopy Experimental cell research High 19331828
2012 The N-terminal domain (NTD) of archaeal Nop56/58 mediates an exceptionally stable interaction with fibrillarin (confirmed by chemical and thermal denaturation). Only deletion of the NTD itself prevented dimerization with fibrillarin. Mutations in the NTD that did not affect fibrillarin binding or sRNP assembly nevertheless disrupted sRNP-guided nucleotide modification, revealing a direct role for Nop56/58 in methyltransferase activity beyond scaffold function. Cross-linking showed Nop56/58 contacts the target RNA substrate. The crystal structure of the Mj Nop56/58 NTD was solved to 1.7 Å, revealing a conserved fold among archaeal homologs despite low primary sequence conservation. Site-directed mutagenesis, chemical/thermal denaturation, in vitro methylation assay, RNA cross-linking, X-ray crystallography (1.7 Å) The Journal of biological chemistry High 22496443
2013 Nol5a/Nop56 was identified as a gene hyperactivated by Burkitt's lymphoma-associated Myc mutants. Nol5a is required for Myc-induced cell transformation: knockdown reduced transformation, while overexpression enhanced MycWT-induced transformation and increased tumor size. This establishes Nop56 as a rate-limiting nucleolar target gene downstream of Myc that links Myc-induced ribosomal RNA methylation to cell transformation. Gene expression profiling, siRNA knockdown, overexpression, cell transformation assay, in vivo tumor growth assay Oncogene Medium 24013231
2018 The intron-hosted box C/D snoRNA snoRD86 acts in cis as a sensor and master switch controlling levels of the limiting snoRNP core protein NOP56. Excess snoRNP core proteins cause snoRD86 to adopt different RNP conformations that dictate usage of nearby alternative splice donors in the NOP56 pre-mRNA, triggering generation of a cytoplasmic snoRD86-containing NOP56-derived lncRNA via the nonsense-mediated decay (NMD) pathway. This constitutes a feedback mechanism based on RNA structure that coordinates box C/D snoRNP core protein levels and global snoRNA levels. RNA splicing analysis, NMD pathway assays, RNP structure analysis, snoRNA expression measurements, alternative splicing mapping Molecular cell High 30220559
2021 High-resolution crystal structure of eukaryotic (yeast) Nop1 (fibrillarin) bound to the amino-terminal domain of Nop56 reveals the protein-protein interface. The eukaryotic Nop56 NTD recruits the methyltransferase to the box C/D RNP through a protein-protein interface that differs substantially from archaeal orthologs, demonstrating evolutionary divergence in the mechanism of methyltransferase recruitment to the C/D RNP complex. X-ray crystallography (high-resolution structure), structural comparison with archaeal orthologs RNA (New York, N.Y.) High 33483369
2022 Zebrafish nop56 loss-of-function mutants exhibit severe neurodegeneration characterized by absence of cerebellum, reduced numbers of spinal cord neurons, high CNS apoptosis, and impaired movement leading to death before 7 days post-fertilization. Gene expression of genes related to the C/D box complex, balance, and CNS development was impaired in mutants, establishing NOP56 as essential for CNS development and neuronal survival in a vertebrate model. Zebrafish loss-of-function genetic model, fluorescence microscopy, apoptosis assays, gene expression analysis, movement assays Biomedicines Medium 36009362
2022 NOP56 depletion causes synthetic lethality with mTOR inhibition in KRAS-mutant lung cancer cells. Mechanistically, NOP56 regulates ROS homeostasis; cells with reduced NOP56 have elevated ROS and rely on mTOR signaling to balance oxidative stress. IRE1α-mediated unfolded protein response (UPR) activates mTOR through p38 MAPK under NOP56 depletion. Co-targeting NOP56 and mTOR profoundly enhances KRAS-mutant tumor cell death in vitro and in vivo. siRNA/shRNA knockdown, CRISPR/Cas9 knockout, flow cytometry (ROS measurement), Western blot, cell viability and apoptosis assays, xenograft tumor model Journal of experimental & clinical cancer research : CR Medium 35039048
2021 NOP56 serves as a negative regulator of the MyD88-mediated NF-κB signaling pathway in teleost fish. NOP56 overexpression inhibited MyD88 protein expression, while siRNA knockdown had the opposite effect. The NOSIC domain of NOP56 is responsible for suppressing MyD88 expression at the protein level. Overexpression, siRNA knockdown, Western blot, domain deletion analysis Fish & shellfish immunology Low 34774735
2025 NOP56 GGCCTG repeat-associated non-AUG (RAN) translation in cell-free systems occurs in all reading frames of the sense strand of NOP56 intron 1. Translation is initiated in a 5' cap-dependent manner from near-cognate start codons upstream of the GGCCUG repeat in each frame. Longer GGCCUG repeats enhance NOP56-RAN translation. A frameshift occurs within the GGCCUG repeat during translation. Cell-free translation systems, in vitro translation assays with repeat constructs of varying lengths, frameshifting analysis The Journal of biological chemistry Medium 40015643
2026 NOP56 interacts with fibrillarin (FBL) in hepatocellular carcinoma cells and activates the PI3K/AKT/CREB pathway. NOP56 knockdown lowered FBL protein levels and suppressed PI3K/AKT/CREB pathway activity, inducing apoptosis and G0/G1 arrest. FBL overexpression partially rescued apoptotic effects of NOP56 silencing, establishing a NOP56-FBL-PI3K/AKT/CREB signaling axis in HCC. Co-immunoprecipitation, Western blot, siRNA knockdown, overexpression, xenograft tumor model, flow cytometry Frontiers in oncology Medium 41568368
2026 NOP56 activates MYC signaling by regulating IRES-dependent translation, which in turn transcriptionally upregulates NOP56 expression creating a positive feedback loop. NOP56 overexpression promotes ribosome biogenesis, cellular proliferation, and metastasis in non-small cell lung cancer. Hypomethylation of the NOP56 promoter contributes to its upregulation in NSCLC. RNA sequencing, qPCR, Western blot, luciferase reporter assay, chromatin immunoprecipitation (ChIP), bisulfite DNA sequencing, functional assays, in vivo tumor model Cancers Medium 41827688
2025 UPR (unfolded protein response) activation significantly downregulates NOP56 expression along with other C/D box snoRNP core proteins (NHP2L1 and FBL). Reduced C/D box snoRNP function during UPR alters rRNA methylation and translational fidelity, including changes in nonsense suppression, frameshifting, ribosome pausing, and IRES-dependent translation initiation. qPCR/Western blot for expression, rRNA methylation assay, translational fidelity reporter assays (nonsense suppression, frameshift, IRES reporters), UPR induction bioRxivpreprint Low

Source papers

Stage 0 corpus · 67 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
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
2015 The BioPlex Network: A Systematic Exploration of the Human Interactome. Cell 1118 26186194
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
2012 The mRNA-bound proteome and its global occupancy profile on protein-coding transcripts. Molecular cell 973 22681889
2005 Nucleolar proteome dynamics. Nature 934 15635413
2020 A reference map of the human binary protein interactome. Nature 849 32296183
2009 A genome-wide RNAi screen identifies multiple synthetic lethal interactions with the Ras oncogene. Cell 843 19490893
2018 VIRMA mediates preferential m6A mRNA methylation in 3'UTR and near stop codon and associates with alternative polyadenylation. Cell discovery 829 29507755
2002 Directed proteomic analysis of the human nucleolus. Current biology : CB 780 11790298
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
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
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
2005 Diversification of transcriptional modulation: large-scale identification and characterization of putative alternative promoters of human genes. Genome research 409 16344560
2015 Panorama of ancient metazoan macromolecular complexes. Nature 407 26344197
2002 Functional proteomic analysis of human nucleolus. Molecular biology of the cell 391 12429849
2007 Systematic analysis of the protein interaction network for the human transcription machinery reveals the identity of the 7SK capping enzyme. Molecular cell 367 17643375
2007 Functional specialization of beta-arrestin interactions revealed by proteomic analysis. Proceedings of the National Academy of Sciences of the United States of America 360 17620599
2011 Expansion of intronic GGCCTG hexanucleotide repeat in NOP56 causes SCA36, a type of spinocerebellar ataxia accompanied by motor neuron involvement. American journal of human genetics 214 21683323
2012 Clinical features of SCA36: a novel spinocerebellar ataxia with motor neuron involvement (Asidan). Neurology 72 22744658
2018 Box C/D snoRNP Autoregulation by a cis-Acting snoRNA in the NOP56 Pre-mRNA. Molecular cell 66 30220559
2020 Chimeric Peptide Species Contribute to Divergent Dipeptide Repeat Pathology in c9ALS/FTD and SCA36. Neuron 60 32375063
2013 Burkitt's lymphoma-associated c-Myc mutations converge on a dramatically altered target gene response and implicate Nol5a/Nop56 in oncogenesis. Oncogene 43 24013231
2020 Hexanucleotide Repeat Expansions in c9FTD/ALS and SCA36 Confer Selective Patterns of Neurodegeneration In Vivo. Cell reports 38 32375043
2007 Alternative conformations of the archaeal Nop56/58-fibrillarin complex imply flexibility in box C/D RNPs. Journal of molecular biology 30 17617422
2006 The coiled-coil domain of the Nop56/58 core protein is dispensable for sRNP assembly but is critical for archaeal box C/D sRNP-guided nucleotide methylation. RNA (New York, N.Y.) 29 16601205
2018 Structural and Dynamical Characterization of DNA and RNA Quadruplexes Obtained from the GGGGCC and GGGCCT Hexanucleotide Repeats Associated with C9FTD/ALS and SCA36 Diseases. ACS chemical neuroscience 24 29281254
2022 Metabolic synthetic lethality by targeting NOP56 and mTOR in KRAS-mutant lung cancer. Journal of experimental & clinical cancer research : CR 23 35039048
2018 Frequency of SCA8, SCA10, SCA12, SCA36, FXTAS and C9orf72 repeat expansions in SCA patients negative for the most common SCA subtypes. BMC neurology 23 29316893
2009 Fibrillarin and Nop56 interact before being co-assembled in box C/D snoRNPs. Experimental cell research 22 19331828
2019 Suppression of the yeast elongation factor Spt4 ortholog reduces expanded SCA36 GGCCUG repeat aggregation and cytotoxicity. Brain research 17 30610877
2018 Nucleolar Division in the Promastigote Stage of Leishmania major Parasite: A Nop56 Point of View. BioMed research international 12 30406129
2015 Spinocerebellar ataxia 36 (SCA36): «Costa da Morte ataxia». Neurologia (Barcelona, Spain) 12 25593102
2020 Cerebellar Cognitive Affective Syndrome in Costa da Morte Ataxia (SCA36). Cerebellum (London, England) 11 32270466
2023 The roles of NOP56 in cancer and SCA36. Pathology oncology research : POR 10 36741964
2022 A nop56 Zebrafish Loss-of-Function Model Exhibits a Severe Neurodegenerative Phenotype. Biomedicines 10 36009362
2013 Early and selective reduction of NOP56 (Asidan) and RNA processing proteins in the motor neuron of ALS model mice. Neurological research 10 23582672
2012 Structurally conserved Nop56/58 N-terminal domain facilitates archaeal box C/D ribonucleoprotein-guided methyltransferase activity. The Journal of biological chemistry 10 22496443
2023 Repeat expansions in NOP56 are a cause of spinocerebellar ataxia Type 36 in the British population. Brain communications 9 37810464
2021 Repeats expansions in ATXN2, NOP56, NIPA1 and ATXN1 are not associated with ALS in Africans. IBRO neuroscience reports 9 34179866
2022 RNA G-quadruplex in live cells lighted-up by a thiazole orange analogue for SCA36 identification. International journal of biological macromolecules 8 36572080
2023 A Chinese SCA36 pedigree analysis of NOP56 expansion region based on long-read sequencing. Frontiers in genetics 6 37051597
2021 NOP56 negatively regulates MyD88-mediated NF-κB signaling in miiuy croaker, Miichthys miiuy. Fish & shellfish immunology 6 34774735
2022 Long-read sequencing identified intronic (GGCCTG)n expansion in NOP56 in one SCA36 family and literature review. Clinical neurology and neurosurgery 5 36368168
2021 High-resolution structure of eukaryotic Fibrillarin interacting with Nop56 amino-terminal domain. RNA (New York, N.Y.) 5 33483369
2025 Dissecting the mechanism of NOP56 GGCCUG repeat-associated non-AUG translation using cell-free translation systems. The Journal of biological chemistry 4 40015643
2002 Characterization of the sequences encoding for Xenopus laevis box C/D snoRNP Nop56 protein. Biochimica et biophysica acta 4 12020815
2025 Structural Insights into an Antiparallel Chair-Type G-Quadruplex From the Intron of NOP56 Oncogene. Advanced science (Weinheim, Baden-Wurttemberg, Germany) 3 40047221
2000 Dnop56, a Drosophila gene homologous to the yeast nucleolar NOP56 gene. Genetica 3 11430491
2024 Hexanucleotide repeat expansion in SCA36 reduces the expression of genes involved in ribosome biosynthesis and protein translation. Journal of human genetics 2 38811808
2022 Identification of Malignancy in PAP Smear Samples Using the CGB3 and NOP56 Genes as Methylation Markers. Asian Pacific journal of cancer prevention : APJCP 2 36308381
2026 NOP56 interacts with Fibrarin to regulate the PI3K/AKT signaling pathway and inhibit apoptosis of hepatocellular carcinoma. Frontiers in oncology 0 41568368
2026 A DNA Methylation-Dependent NOP56/MYC Positive Feedback Loop Promotes the Proliferation and Migration of Non-Small Cell Lung Cancer Through Regulating Ribosome Biogenesis. Cancers 0 41827688
2025 A fly model of SCA36 reveals combinatorial neurotoxicity of hexanucleotide and dipeptide repeats. PLoS genetics 0 41337098
2024 Investigating Repeat Expansions in NIPA1, NOP56, and NOTCH2NLC Genes: A Closer Look at Amyotrophic Lateral Sclerosis Patients from Southern Italy. Cells 0 38667292