Affinage

NEU1

Sialidase-1 · UniProt Q99519

Length
415 aa
Mass
45.5 kDa
Annotated
2026-06-10
100 papers in source corpus 39 papers cited in narrative 39 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

NEU1 is a sialidase that catabolizes sialylated glycoconjugates in lysosomes and, when relocalized to the plasma membrane, desialylates a defined set of cell-surface glycoproteins to control receptor signaling across immunity, inflammation, vascular biology, and tissue remodeling (PMID:19796680, PMID:19430901, PMID:16835219, PMID:22403397). Its structure reveals self-association interfaces and a catalytic loop that adopts an inactive conformation, and stable heterodimerization with the chaperone PPCA/cathepsin A both reverses NEU1 self-oligomerization and provides the conformational activation needed for catalysis and lysosomal targeting (PMID:37205763, PMID:19666471). At the cell surface, NEU1 acts within multiprotein platforms: it forms a complex with TLR2/3/4 and removes alpha-2,3-sialyl residues from TLR4, relieving steric hindrance to receptor dimerization and MyD88 recruitment and de-repressing TLR4 from inhibitory Siglec interaction to drive NFκB activation (PMID:19796680, PMID:19430901, PMID:25187624). This sialidase activity is gated by a GPCR–MMP-9 cascade, in which ligand engagement of neuromedin B receptor or TrkA triggers Gαi and MMP-9 activation that in turn activates NEU1 (PMID:21873432, PMID:20347965, PMID:23827939, PMID:22759791). NEU1 desialylates a broad substrate repertoire—integrin β4, CD31, EGFR, MUC1, CD44, CD5, CD36, β2 integrin, and the viral coronavirus nucleocapsid—to modulate FAK/ERK, PI3K, and downstream signaling that governs cancer cell migration and metastasis, angiogenesis, monocyte adhesion, and viral replication (PMID:19151752, PMID:22247545, PMID:24550400, PMID:25963144, PMID:36714013, PMID:34903296, PMID:29897583). NEU1 substrate engagement is spatially controlled, requiring homophilic CD31 engagement and a Src-kinase/p120-catenin adaptor bridge to couple NEU1 to its target (PMID:28343945, PMID:24550400). Beyond catalysis, NEU1 acts as a non-enzymatic scaffold, binding the MUC1 cytoplasmic domain to block PI3K–Akt signaling independently of its sialidase activity (PMID:34688655). NEU1 also negatively regulates lysosomal exocytosis, and its loss drives pathological exocytosis underlying amyloidogenic APP processing in Alzheimer-model brain and a tumor-suppressor phenotype in rhabdomyosarcoma (PMID:24225533, PMID:36127469). Loss-of-function NEU1 mutations cause type II sialidosis (PMID:14695530).

Mechanistic history

Synthesis pass · year-by-year structured walk · 39 steps
  1. 1998 High

    Establishing that a single residue controls NEU1 catalytic competence without disrupting trafficking or chaperone binding linked enzyme structure to enzymatic deficiency.

    Evidence Site-directed mutagenesis, enzyme activity assays, lysosomal fractionation and PPCA binding in SM/J mice

    PMID:9425240

    Open questions at the time
    • Does not resolve the atomic basis of altered substrate affinity
    • Tested a single naturally occurring variant
  2. 2004 High

    Mapping disease mutations to distinct mechanistic defects established NEU1 loss-of-function as causative for type II sialidosis.

    Evidence Adenoviral expression of patient mutant alleles, sialidase activity and localization assays

    PMID:14695530

    Open questions at the time
    • Folding-vs-substrate-binding assignment is inferential without structural data
    • Genotype–phenotype severity correlations not addressed
  3. 2005 Medium

    Linking NEU1 induction to MyoD-driven myogenic differentiation showed that regulated sialidase activity is required for proper differentiation timing.

    Evidence C2C12 differentiation with MyoD reporter and NEU1 overexpression, myogenic marker Westerns

    PMID:16216242

    Open questions at the time
    • Relevant NEU1 substrates in myoblasts not identified
    • Single cell line
  4. 2006 High

    Demonstrating NEU1 relocalization from lysosome to plasma membrane during macrophage differentiation revealed a surface pool with immune function.

    Evidence Immunofluorescence, fractionation, siRNA and antibody blocking with phagocytosis/cytokine readouts

    PMID:16835219

    Open questions at the time
    • Trafficking machinery for surface delivery not fully defined
    • Surface substrates not yet identified in this study
  5. 2009 High

    Defining NEU1–PPCA heterodimerization clarified how premature self-oligomerization is prevented and NEU1 is activated and trafficked.

    Evidence Analytical ultracentrifugation, binding-site mapping, biochemical reconstitution

    PMID:19666471

    Open questions at the time
    • High-resolution structure of the heterodimer not resolved here
    • Dynamics of oligomer disassembly in vivo not measured
  6. 2009 High

    Identifying the NEU1–TLR4 complex and TLR4 desialylation as a requirement for TLR4 dimerization and NFκB activation established NEU1 as a positive regulator of innate immune signaling.

    Evidence Reciprocal co-IP, live-cell sialidase assay, Neu1-deficient macrophages, catalytic mutant, pharmacological inhibition

    PMID:19430901 PMID:19796680

    Open questions at the time
    • Precise sialoglycan sites on TLR4 not mapped
    • Mechanism of NEU1 surface activation not addressed in this study
  7. 2009 High

    Showing NEU1 desialylates integrin β4 to suppress FAK/ERK signaling and metastasis positioned NEU1 as a regulator of cancer cell invasion.

    Evidence NEU1 overexpression/knockdown, lectin blotting of integrin β4, in vivo transsplenic metastasis model

    PMID:19151752

    Open questions at the time
    • Whether surface NEU1 acts within a defined complex on integrin β4 unknown
    • Single tumor model
  8. 2009 Medium

    Identifying an Nkx3-binding promoter mutation explained reduced Neu1 expression and immune phenotypes in SM/J mice.

    Evidence Reporter assays, recombinant Nkx3.2 binding/repression, intravital microcirculation imaging

    PMID:19217813

    Open questions at the time
    • Relevance to human NEU1 regulation not established
    • In vivo TF occupancy not directly shown
  9. 2007 High

    Demonstrating that the NEU1 subunit of the elastin receptor complex drives ERK1/2 and pro-MMP-1 responses established NEU1 catalysis as the effector of elastin-peptide signaling.

    Evidence Catalytic-dead mutant, siRNA, ERK1/2 and pro-MMP-1 assays in fibroblasts

    PMID:17327233

    Open questions at the time
    • Direct desialylation substrate within the ERC pathway not identified here
    • Single cell type
  10. 2010 Medium

    Extending the GPCR–MMP-9–NEU1 module to TrkA/NGF signaling generalized the surface activation cascade beyond TLR4.

    Evidence Co-IP, live-cell sialidase assay, Tamiflu inhibition, neurite outgrowth in TrkA-PC12 cells

    PMID:20347965

    Open questions at the time
    • Direct TrkA desialylation not biochemically demonstrated
    • Single lab
  11. 2010 Medium

    Linking NEU1 to CD44 desialylation in CD4+ T cells connected NEU1 to HA-receptor function and Th2 airway inflammation.

    Evidence HA-binding flow cytometry, sialidase inhibition, Neu1-deficient SM/J mice, airway hyperresponsiveness

    PMID:20491786

    Open questions at the time
    • CD44 substrate inferred rather than shown by immunoprecipitation
    • Indirect effects of global Neu1 deficiency not excluded
  12. 2011 High

    Placing MMP-9 between Gαi-GPCR signaling and NEU1 activation defined the molecular order of TLR4-coupled sialidase induction.

    Evidence Co-IP, MMP-9 KO macrophages, shRNA/siRNA, live-cell sialidase assay, pharmacological inhibition

    PMID:21873432

    Open questions at the time
    • Direct mechanism by which MMP-9 activates NEU1 not resolved
    • Stoichiometry of the TLR4–MMP9–NEU1 complex unknown
  13. 2012 High

    Identifying EGFR and MUC1 as NEU1-associated substrates in airway epithelium showed NEU1 restrains EGFR autophosphorylation and modulates host–pathogen interactions.

    Evidence Co-IP, NEU1 overexpression/siRNA, lectin blotting, EGFR phosphorylation and bacterial adhesion assays

    PMID:22247545

    Open questions at the time
    • Direct desialylation sites on EGFR not mapped
    • In vivo relevance not tested in this study
  14. 2012 High

    Quantifying NEU1 as the dominant endothelial sialidase that restrains migration tied NEU1 expression level to vascular cell behavior.

    Evidence RT-PCR, Western, confocal localization, siRNA, wound-healing and activity assays

    PMID:22403397

    Open questions at the time
    • Endothelial substrates not identified in this study
    • Mechanism of perinuclear vs surface pools unclear
  15. 2012 Medium

    Generalizing NMBR-MMP9-NEU1 to multiple GPCR agonists established a broad transactivation platform for TLR signaling.

    Evidence Live-cell sialidase assay, siRNA, co-IP, NFκB reporter, Neu1-deficient macrophages

    PMID:22759791

    Open questions at the time
    • Direct receptor desialylation events not all demonstrated
    • Single lab
  16. 2013 High

    Connecting NEU1 deficiency to oversialylated APP, lysosomal exocytosis, and Aβ release, with rescue by NEU1 injection, implicated NEU1 in amyloid pathology.

    Evidence NEU1-deficient mice, APP/Aβ analysis, lysosomal fractionation, cerebral NEU1 rescue

    PMID:24225533

    Open questions at the time
    • Direct desialylation of APP by NEU1 not shown biochemically
    • Human relevance not established
  17. 2013 Medium

    Extending the NMBR-MMP9-Neu1 platform to endosomal TLR7/9 broadened NEU1's role to nucleic-acid sensing.

    Evidence siRNA of complex components, co-IP, NFκB reporter, cytokine readouts

    PMID:23827939

    Open questions at the time
    • Endosomal substrate of NEU1 not identified
    • Single lab
  18. 2013 Medium

    Demonstrating cathepsin A–NEU1–dependent PI3Kγ signaling in monocytes linked the elastin receptor complex to atherosclerotic plaque progression.

    Evidence Atherosclerosis mouse models, hematopoietic-specific complex deletion, neuraminidase inhibition, migration/ROS assays

    PMID:24357053

    Open questions at the time
    • Direct NEU1 substrate upstream of PI3Kγ not defined here
    • Catalysis-vs-scaffold contribution not separated
  19. 2014 High

    Identifying CD31 as a NEU1 substrate whose desialylation inhibits angiogenesis established a catalysis-dependent anti-angiogenic role.

    Evidence Catalytic-dead G68V mutant, NEU1/CD31 knockdown, CD31-null cells, lectin blotting, tube formation assay

    PMID:24550400

    Open questions at the time
    • Spatial trigger for CD31 desialylation not yet defined in this study
    • In vivo angiogenesis not tested
  20. 2014 High

    Showing NEU1 disrupts inhibitory TLR4:Siglec-E interactions defined a positive-feedback de-repression mechanism for TLR activation in vivo.

    Evidence Surface translocation assay, sialidase inhibitor, conditional hematopoietic Neu1 KO, endotoxemia model

    PMID:25187624

    Open questions at the time
    • Quantitative kinetics of Siglec disengagement not measured
    • Other Siglec partners not surveyed
  21. 2015 High

    Defining NEU1-driven MUC1 ectodomain desialylation and shedding revealed a decoy-receptor mechanism in bacterial defense.

    Evidence Scatchard binding, NEU1/MUC1 co-IP, lectin blotting, adhesion/invasion assays, ex vivo BAL fluid

    PMID:25963144

    Open questions at the time
    • In vivo contribution to infection outcome not tested here
    • Role of PPCA co-association mechanistically unresolved
  22. 2016 Medium

    Showing NEU1 overexpression recapitulates lung fibrosis features positioned NEU1 as a driver of fibrotic tissue remodeling.

    Evidence Adenoviral NEU1 delivery in vitro/in vivo, collagen/MMP-14 assays, T-cell adhesion, BAL analysis

    PMID:26993524

    Open questions at the time
    • Causal substrates underlying fibrosis not identified
    • Overexpression may not reflect physiologic levels
  23. 2017 High

    Identifying the SFK/p120-catenin adaptor bridge explained how NEU1 is spatially coupled to CD31 only under confluence.

    Evidence Co-IP, siRNA of SFKs/CD31/p120ctn, SFK inhibitors, cell-free reconstitution with purified proteins

    PMID:28343945

    Open questions at the time
    • Whether similar adaptors gate other NEU1 substrates unknown
    • Stoichiometry of the assembled scaffold not determined
  24. 2017 Medium

    Demonstrating NEU1 interaction with Plin1 on lipid droplets identified a non-immune role restraining lipolysis.

    Evidence siRNA, co-IP, co-localization, glycerol release and Plin1 phosphorylation assays in adipocytes

    PMID:28429532

    Open questions at the time
    • Whether the effect is catalytic or scaffold not resolved
    • Single cell model
  25. 2017 Medium

    Showing GPCR agonists activate NEU1 and insulin-receptor signaling via NMBR-MMP9-Neu1 extended the platform to metabolic receptor crosstalk.

    Evidence Co-IP of AT1R–Neu1–IRβ–NMBR complex, live-cell sialidase assay, IR phosphorylation, pharmacological inhibition

    PMID:29277445

    Open questions at the time
    • Direct IR desialylation not demonstrated
    • Single lab
  26. 2018 Medium

    Identifying CD36 as a NEU1 substrate downstream of the ERC linked NEU1 to macrophage oxLDL uptake and foam-cell biology.

    Evidence LC-MS/MS, co-IP, sialidase assay, oxLDL uptake assay with pharmacological controls

    PMID:30498996

    Open questions at the time
    • CD36 desialylation sites not mapped
    • Single lab
  27. 2018 Medium

    Establishing CD5 as a cell-surface NEU1 substrate on thymocytes added a lymphocyte target to the NEU1 repertoire.

    Evidence PNA-blot of anti-CD5 IP, NEU1-selective inhibitor, SM/J-deficient comparison

    PMID:29897583

    Open questions at the time
    • Functional consequence of CD5 desialylation not defined
    • Single lab
  28. 2021 High

    Discovering a catalysis-independent scaffold function at the MUC1 cytoplasmic domain showed NEU1 can inhibit PI3K–Akt signaling without sialidase activity.

    Evidence Reciprocal co-IP, GST pull-down deletion mapping, cell-free binding with purified proteins, G68V catalytic-dead mutant

    PMID:34688655

    Open questions at the time
    • Structural basis of the NEU1–MUC1-CD interaction unknown
    • Generality of scaffold mode across substrates unclear
  29. 2021 Medium

    Showing CD31 desialylation requires homophilic CD31 engagement refined the spatial logic of NEU1 substrate selection.

    Evidence siRNA, WT and catalytic-dead G68V overexpression, lectin blotting, tube formation/migration assays

    PMID:24550400

    Open questions at the time
    • Conformational sensing mechanism not defined
    • Single lab extension
  30. 2021 Medium

    Identifying β2 integrin and ICAM-1 as ERC-downstream NEU1 substrates linked NEU1 to monocyte adhesion and transendothelial migration.

    Evidence Proteomics, co-IP, sialidase assay, adhesion and transmigration assays

    PMID:34903296

    Open questions at the time
    • In vivo relevance not established
    • Single lab
  31. 2021 Medium

    Showing pathogen-induced suppression of Neu1 phosphorylation/translocation defined a host-evasion mechanism via TLR4 hypersialylation.

    Evidence IP, phosphorylation analysis, NEU1 overexpression/silencing, confocal, cytokine ELISA in Leishmania-infected macrophages

    PMID:33763070

    Open questions at the time
    • Kinase responsible for Neu1 phosphorylation not identified
    • Single lab
  32. 2022 Medium

    Demonstrating that secreted active Neu1 from microglia desialylates Trem2/MerTK and neurons connected NEU1 to phagocytosis and neuronal vulnerability.

    Evidence Neu1 knockdown/overexpression, exocytosis inhibitors, phagocytosis and co-IP, galectin-3 binding, neuronal toxicity assays

    PMID:35693885

    Open questions at the time
    • In vivo relevance to neurodegeneration not established
    • Single lab
  33. 2022 Medium

    Placing NEU1 upstream of the SIRT1/PGC-1α axis defined a role in post-MI cardiac metabolism and oxidative stress.

    Evidence Cardiomyocyte-specific NEU1 KO, SIRT1 inhibitor, PGC-1α KO epistasis, echocardiography and metabolic assays

    PMID:35548408

    Open questions at the time
    • Molecular link between NEU1 and SIRT1/PGC-1α not defined
    • Single lab
  34. 2022 Medium

    Showing NEU1 haploinsufficiency increases lysosomal exocytosis and drives rhabdomyosarcoma established a tumor-suppressor function via exocytosis control.

    Evidence NEU1 haploinsufficient mouse model, LAMP1 redistribution, single-cell exocytosis analysis

    PMID:36127469

    Open questions at the time
    • Substrate driving exocytosis suppression not identified
    • Single model
  35. 2023 High

    The first 3D structure of NEU1 revealed self-association interfaces and an inactive catalytic loop, providing a structural model for PPCA-dependent activation.

    Evidence Crystal/cryo-EM structure determination and biochemical characterization of murine NEU1

    PMID:37205763

    Open questions at the time
    • Structure of the activated PPCA-bound state not resolved
    • Human NEU1 structure inferred from murine
  36. 2023 Medium

    Identifying ATG5 as a hypoxia-dependent NEU1 substrate connected NEU1 to autophagosome formation and inflammatory responses.

    Evidence Co-IP, NEU1 knockdown/inhibition, ATG5 sialylation and complex-formation analysis, autophagosome assay

    PMID:37844713

    Open questions at the time
    • Whether ATG5 sialylation occurs at the lysosome or elsewhere unclear
    • Single lab
  37. 2023 Medium

    Showing NEU1 controls sialylation of the coronavirus nucleocapsid to regulate replication identified a viral substrate and antiviral target.

    Evidence NEU1 overexpression/knockdown, NEU1 inhibitor, nucleocapsid sialylation and RNA-binding assays, in vivo HCoV-OC43 rescue

    PMID:36714013

    Open questions at the time
    • Mechanism of nucleocapsid sialylation/desialylation balance unclear
    • Single lab
  38. 2024 Medium

    Demonstrating fibroblast-derived NEU1 keeps osteoclasts mononuclear linked NEU1 to cortical bone metabolism and fracture healing.

    Evidence scRNA-seq of human periosteum, NEU1 inhibition in vitro/in vivo, osteoclast fusion analysis, fracture healing model

    PMID:41356197

    Open questions at the time
    • Specific osteoclast glycoprotein substrate not identified
    • Single lab
  39. 2025 Medium

    Showing epididymal cells transfer NEU1 to sperm to regulate α-2,6 sialylation tied NEU1 to sperm maturation and fertility.

    Evidence Immunofluorescence, Western, NEU1 inhibition, IVF, murine transfer model, clinical correlation

    PMID:41329355

    Open questions at the time
    • Direct sperm-surface substrate not identified
    • Single lab

Open questions

Synthesis pass · forward-looking unresolved questions
  • How NEU1 substrate selectivity is encoded—distinguishing catalytic versus scaffold modes and the structural basis of activation in the PPCA-bound state—remains unresolved.
  • No structure of the activated human PPCA-NEU1 complex
  • General rules governing which substrates require adaptor bridges vs direct association not defined
  • Relative physiologic contribution of lysosomal vs surface vs secreted NEU1 pools unclear

Mechanism profile

Synthesis pass · controlled-vocabulary classification · explore literature graph →
Molecular activity
GO:0140098 catalytic activity, acting on RNA 7 GO:0016787 hydrolase activity 4 GO:0098772 molecular function regulator activity 3 GO:0060090 molecular adaptor activity 2
Localization
GO:0005886 plasma membrane 5 GO:0005764 lysosome 4 GO:0005576 extracellular region 2 GO:0005811 lipid droplet 1
Pathway
R-HSA-168256 Immune System 5 R-HSA-162582 Signal Transduction 4 R-HSA-8953854 Metabolism of RNA 2 R-HSA-9609507 Protein localization 2 R-HSA-9612973 Autophagy 1
Complex memberships
NEU1-PPCA/cathepsin A heterodimerNMBR-MMP9-NEU1 GPCR signaling platformTLR4-MMP-9-NEU1 surface complexelastin receptor complex (ERC)

Evidence

Reading pass · 39 per-paper findings extracted from the source corpus
Year Finding Method Journal Conf PMIDs
2023 Crystal/cryo-EM structure of murine NEU1 was determined, revealing that the enzyme oligomerizes through two self-association interfaces, displays a wide substrate-binding cavity, and contains a catalytic loop that adopts an inactive conformation. A mechanism of activation was proposed involving a conformational change in this loop upon binding to its protective protein PPCA/cathepsin A. Structure determination (3D structural analysis), biochemical characterization Science advances High 37205763
2009 NEU1 (and not NEU2, NEU3, or NEU4) forms a complex with TLR-2, -3, and -4 on the cell surface of naïve macrophages. Ligand binding to TLR4 (LPS) induces NEU1 sialidase activity, which hydrolyzes alpha-2,3-sialyl residues on TLR4, removing steric hindrance to TLR4 dimerization, MyD88/TLR4 complex formation, and subsequent NFκB activation. This was confirmed using primary Neu1-deficient macrophages (which fail to respond), catalytically inactive trans-sialidase mutant (which cannot substitute), and the neuraminidase inhibitor Tamiflu. Co-immunoprecipitation, live-cell sialidase assay, pharmacological inhibition (Tamiflu/DANA/zanamivir), lectin blocking, primary Neu1-deficient macrophages, HEK293 reconstitution with TLR4/MD2 Cellular signalling High 19430901 19796680
2009 NEU1 forms a stable heterodimer with protective protein/cathepsin A (PPCA). PPCA acts as a molecular chaperone that prevents premature self-oligomerization of NEU1 by competing for a NEU1 self-association interface. In the absence of PPCA (as in galactosialidosis), NEU1 self-associates into chain-like oligomers. PPCA binding reverses NEU1 self-oligomerization by disassembling NEU1 oligomers into a PPCA-NEU1 heterodimer. Hydrodynamic analysis (analytical ultracentrifugation), binding site mapping, structural modeling, biochemical reconstitution The Journal of biological chemistry High 19666471
2006 During monocyte-to-macrophage differentiation, NEU1 relocalizes from lysosomes to the cell surface via LAMP-2-negative, MHC class II-positive vesicles that merge with the plasma membrane. Cathepsin A, which forms a complex with and activates NEU1 in lysosomes, is co-sorted to the plasma membrane. siRNA suppression of NEU1 or anti-NEU1 antibodies reduced macrophage phagocytosis of bacteria and cytokine production. Immunofluorescence, subcellular fractionation, siRNA knockdown, anti-Neu1 antibody blocking, flow cytometry The Journal of biological chemistry High 16835219
2007 The catalytic sialidase activity of the NEU1 subunit of the elastin receptor complex (ERC) is responsible for ERK1/2 activation and pro-MMP-1 production induced by elastin peptide binding. A catalytically inactive NEU1 mutant and siRNA-mediated NEU1 knockdown both abolished elastin peptide-induced ERK1/2 activation and pro-MMP-1 production in skin fibroblasts. N-acetyl neuraminic acid (sialic acid) could reproduce the elastin peptide effects. Pharmacological inhibition, catalytically inactive NEU1 mutant, siRNA knockdown, ERK1/2 activation assay, pro-MMP-1 production assay The Journal of biological chemistry High 17327233
2011 On the surface of naive macrophage cells, NEU1 forms a complex with MMP-9, both of which are constitutively bound to TLR4. LPS binding to TLR4 activates Gαi GPCR signaling, which activates MMP-9, which in turn activates NEU1 sialidase. NEU1-mediated TLR4 desialylation and NFκB activation are blocked by specific MMP-9 inhibition, Gαi-pathway inhibition, MMP-9 shRNA/siRNA knockdown, and are absent in MMP-9 knockout macrophages. Co-immunoprecipitation, siRNA/shRNA knockdown, MMP-9 KO macrophages, lentiviral transduction, pharmacological inhibition, live-cell sialidase assay The Journal of biological chemistry High 21873432
2010 Neu1 and MMP-9 form a complex bound to TrkA on the surface of naive neurons and TrkA-expressing cells. NGF binding to TrkA activates GPCR Gαi signaling → MMP-9 activation → Neu1 sialidase activation. Tamiflu (oseltamivir phosphate) blocks NGF-induced Neu1 activity and subsequent Trk receptor activation and neurite outgrowth in TrkA-PC12 cells. Co-immunoprecipitation, live-cell sialidase assay, pharmacological inhibition (Tamiflu), TrkA-PC12 cell lines, primary neurons Cellular signalling Medium 20347965
2009 NEU1 overexpression in human colon cancer HT-29 cells desialylates integrin beta4 (removing alpha-2,3 and O-glycan sialic acids), reduces integrin beta4 phosphorylation, attenuates focal adhesion kinase (FAK) and Erk1/2 signaling, and downregulates MMP-7, leading to suppressed cell migration, invasion, adhesion, and in vivo liver metastasis. NEU1 overexpression and siRNA knockdown, lectin blotting of integrin beta4 immunoprecipitates, immunofluorescence, biotinylation assay, O-glycosylation inhibitor (GalNAc-α-O-benzyl), in vivo transsplenic injection model Oncogene High 19151752
2012 In human airway epithelial cells, NEU1 sialidase directly associates with EGFR and MUC1 and accounts for >70% of cellular sialidase activity. NEU1 overexpression diminishes EGF-stimulated EGFR Tyr-1068 autophosphorylation, while NEU1 depletion increases EGFR activation. NEU1 also desialylates MUC1, enhancing MUC1-dependent Pseudomonas aeruginosa adhesion and flagellin-stimulated ERK1/2 activation. Co-immunoprecipitation, NEU1 overexpression/siRNA, lectin blotting (MAL-2/PNA), EGFR phosphorylation assay, bacterial adhesion assay The Journal of biological chemistry High 22247545
2013 NEU1 deficiency in mice leads to accumulation and amyloidogenic processing of an oversialylated amyloid precursor protein (APP) in lysosomes, followed by excessive lysosomal exocytosis that releases Aβ peptides extracellularly. Cerebral injection of NEU1 into an established AD mouse model substantially reduces β-amyloid plaques. NEU1-deficient mouse model, APP processing analysis, lysosomal fractionation, Aβ ELISA, cerebral NEU1 injection rescue experiment Nature communications High 24225533
2014 TLR4 activation triggers NEU1 translocation to the cell surface, where NEU1 disrupts the inhibitory TLR4:Siglec-E interaction by desialylating TLR4. Sialidase inhibitor Neu5Gc2en prevents TLR4 ligand-induced disruption of TLR4:Siglec-E/F interactions. Absence of NEU1 in hematopoietic cells protects mice against endotoxemia, demonstrating that NEU1-mediated de-repression of TLR4 from Siglec inhibition is a positive feedback mechanism for TLR activation during infection. Cell surface translocation assay, sialidase inhibitor treatment (Neu5Gc2en), Neu1-conditional hematopoietic KO mice, TLR4:Siglec interaction co-IP/pulldown, endotoxemia mouse model eLife High 25187624
2014 Catalytically active NEU1 inhibits in vitro angiogenesis (capillary-like tube formation) through desialylation of its substrate CD31 in postconfluent human pulmonary microvascular endothelial cells. A catalytically dead NEU1 mutant (G68V) did not inhibit tube formation. Prior CD31 silencing or use of CD31-null ECs abrogated NEU1's inhibitory effect, and forced CD31 sialylation (via sialyltransferase ST6GAL-I overexpression) counteracted NEU1-mediated inhibition. NEU1 overexpression with catalytic mutant control (G68V), siRNA knockdown of NEU1/CD31, CD31-null ECs, lectin blotting (SNA/PNA), Matrigel tube formation assay, wounding assay The Journal of biological chemistry High 24550400
1998 A single amino acid substitution L209I in the Neu1 protein is responsible for the partial neuraminidase deficiency in SM/J mice. The mutant enzyme is correctly localized to lysosomes and retains the ability to associate with its activating protein PPCA, but the reduced activity is caused by altered substrate affinity rather than changed substrate specificity or turnover rate. Genetic analysis, sequencing, site-directed mutagenesis, enzyme activity assays, lysosomal fractionation, PPCA binding assay Human molecular genetics High 9425240
2004 Five novel NEU1 mutations (R225P, A298V, M1?, R341G, W23X) in type II sialidosis patients abolish enzymatic sialidase activity when expressed via recombinant adenovirus. The R341G mutation perturbs substrate binding, while A298V and R225P impair enzyme folding, as determined by activity assay and immunofluorescence localization analysis. Adenoviral expression of mutant sialidases in primary cell cultures, sialidase activity assay, intracellular localization (immunofluorescence) Human mutation High 14695530
2012 NEU1 is the dominant sialidase in human lung microvascular endothelial cells (expressed ~2700-fold more than NEU2/3/4), localizes to both plasma membrane and perinuclear regions, and restrains endothelial cell migration. NEU1 overexpression reduced EC migration into wounds by >40%, whereas NEU3 overexpression did not. siRNA knockdown of NEU1 decreased >65% of total sialidase activity for 4-MU-NANA substrate. Real-time RT-PCR, Western blot, confocal microscopy/flow cytometry (localization), siRNA knockdown, wound-healing migration assay, sialidase activity assay The Journal of biological chemistry High 22403397
2010 Neu1 sialidase is required for hyaluronic acid receptor function of CD44 on CD4+ T cells. Antigen stimulation induces Neu1 expression on splenic CD4+ T cells from asthmatic mice, and increased Neu1 activity removes inhibitory sialic acid residues from CD44 to enable HA binding and T helper type 2-mediated airway inflammation. Sialidase inhibitor treatment suppressed HA binding, and Neu1-deficient SM/J mice showed reduced Th2 airway inflammation and airway hyperresponsiveness. Flow cytometry (HA-binding), real-time RT-PCR, pharmacological sialidase inhibition, Neu1-deficient SM/J mouse model, cytokine analysis, airway hyperresponsiveness measurement Clinical and experimental immunology Medium 20491786
2013 NEU1 and MMP-9 form a tripartite complex with neuromedin B GPCR, TLR-7, and TLR-9. Ligand-induced activation of endosomal TLR-7 and TLR-9 requires this NMBR-MMP9-Neu1 cross-talk signaling platform. siRNA silencing of Neu1, MMP-9, or NMBR each significantly reduced nucleic acid-induced NFκB activation and TNFα/MCP-1 cytokine responses. siRNA knockdown, complex formation (immunoprecipitation), NFκB reporter assay, SEAP reporter, pharmacological inhibitors (Tamiflu, MMP-9 inhibitor, BIM23127, BIM-46174) Cellular signalling Medium 23827939
2012 GPCR agonists (bombesin, bradykinin, LPA, cholesterol, angiotensin-1 and -2) induce Neu1 sialidase activity in macrophages via a neuromedin B receptor (NMBR)-MMP9 signaling platform, transactivating TLR receptors and inducing NFκB activation. Bombesin receptor (NMBR) forms a complex with TLR4 and MMP9. MMP9 siRNA knockdown or MMP9 absence abolishes this GPCR-induced Neu1 activity. Live-cell sialidase assay, siRNA knockdown, co-immunoprecipitation, NFκB-SEAP reporter, pharmacological inhibitors, Neu1-deficient primary macrophages as negative control Cellular signalling Medium 22759791
2013 Elastin-derived peptides (EDP) binding to the elastin receptor complex (ERC, which contains NEU1) activate monocyte migration and ROS production through a neuraminidase activity-dependent PI3Kγ signaling pathway. The absence of the cathepsin A-NEU1 complex in hematopoietic cells abolished atherosclerotic plaque progression in LDLR-/- mice. In vitro, PI3Kγ was required for EDP-induced monocyte migration and this was dependent upon neuraminidase activity. In vivo atherosclerosis mouse models, PI3Kγ KO bone marrow chimeras, cathepsin A-NEU1 complex-deficient hematopoietic cells, neuraminidase activity inhibition, monocyte migration assay, ROS production assay Cardiovascular research Medium 24357053
2015 NEU1-driven MUC1 ectodomain desialylation increases binding affinity of P. aeruginosa flagellin to MUC1, promotes bacterial adhesion and invasion, and triggers MUC1-ED shedding. The shed desialylated MUC1-ED functions as a hyperadhesive decoy receptor that competitively blocks bacterial adhesion. NEU1 association with MUC1 increases upon flagellin stimulation, and NEU1 co-associates with MUC1's chaperone PPCA in this context. Scatchard binding analysis, NEU1/MUC1 co-immunoprecipitation, lectin blotting, NEU1 overexpression/siRNA, bacterial adhesion/invasion assay, ex vivo BAL fluid analysis The Journal of biological chemistry High 25963144
2021 NEU1 directly associates with the MUC1 cytoplasmic domain (CD) at its juxtamembranous 36 amino acid region, independently of NEU1 catalytic activity and independently of PPCA chaperone. This NEU1-MUC1-CD interaction does not require NEU1 enzymatic activity for MUC1-ED desialylation. However, both wild-type NEU1 and the catalytically dead NEU1-G68V mutant inhibit PI3K binding to MUC1-CD and reduce downstream Akt phosphorylation, indicating NEU1 inhibits PI3K-Akt signaling through its scaffold function independent of catalytic activity. Reciprocal co-immunoprecipitation, in vitro binding assays (GST pull-down with deletion mutants), cell-free binding assay with purified proteins, catalytic dead mutant (G68V), NEU1-selective inhibitor C9-BA-DANA The Journal of biological chemistry High 34688655
2017 As human pulmonary microvascular endothelial cells (HPMECs) achieve confluence, Src family kinases (SFKs) are activated and phosphorylate p120 catenin. Tyrosine-phosphorylated p120 catenin functions as an adaptor molecule that physically couples NEU1 to CD31, enabling NEU1-mediated CD31 desialylation. SFK inhibition blocks both p120ctn phosphorylation and NEU1-CD31 association. Direct protein-protein interactions between NEU1, CD31, and p120ctn were demonstrated in a cell-free system with purified recombinant proteins. Co-immunoprecipitation, siRNA knockdown of individual SFKs/CD31/p120ctn, SFK pharmacological inhibitors (PP2, SU6656), cell-free binding assay with purified recombinant proteins, pull-down assays Cellular signalling High 28343945
2018 Membrane NEU1 interacts with CD36 (class B scavenger receptor) in human macrophages. Elastin-derived peptides (EDP) binding to the elastin receptor complex desialylate CD36 via NEU1. EDP-induced desialylation of CD36 increases uptake of oxidized LDL by macrophages, an effect blocked by both V14 peptide (blocking EDP-ERC interaction) and the sialidase inhibitor DANA. Proteomic purification (LC-MS/MS), co-immunoprecipitation validation, sialidase activity assay, oxidized LDL uptake assay, pharmacological inhibition (V14 peptide, DANA) Cellular and molecular life sciences Medium 30498996
2021 In L. donovani-infected macrophages, Neu1 translocation to the plasma membrane is impaired due to reduced tyrosine-phosphorylation of Neu1 and diminished Neu1-cathepsin A association. Reduced membrane-bound Neu1 results in hypersialylated TLR4 (enhanced alpha-2,3-linked sialic acids), reduced Neu1-TLR4 association, impaired MyD88 recruitment, and suppressed downstream MAP kinase/NFκB signaling. NEU1 overexpression rescued TLR4 desialylation, MyD88 association, and cytokine production, reducing parasite burden. Immunoprecipitation, phosphorylation analysis, NEU1 overexpression, NEU1 silencing, flow cytometry, confocal microscopy, ELISA cytokine assay Frontiers in immunology Medium 33763070
2005 Lysosomal sialidase (Neu1) activity is induced ~6-fold in the first 24 h of C2C12 myoblast differentiation, driven by MyoD transcriptional activation through a mechanism dependent on MyoD's chromatin remodeling domain. Inappropriate Neu1 overexpression 48 h after differentiation onset downregulates myogenin and myosin heavy chain expression and halts the myogenic differentiation cascade. C2C12 myoblast differentiation, sialidase activity assay, MyoD overexpression/reporter assay, Neu1 overexpression, Western blot (myogenin/MHC) Experimental cell research Medium 16216242
2022 Activated microglia release Neu1 into culture medium by lysosomal exocytosis (blocked by lysosomal exocytosis inhibitors). Released/extracellular Neu1 and Neu1 overexpression increase microglial phagocytosis, while Neu1 knockdown decreases phagocytosis. Microglial activation desialylates phagocytic receptors Trem2 and MerTK via Neu1, increasing Trem2 ligand (galectin-3) binding. Conditioned medium from activated microglia containing Neu1 desialylates neurons and sensitizes them to glutamate-induced death. Neu1 knockdown, exocytosis inhibitor treatment, Neu1 overexpression, phagocytosis assay, co-immunoprecipitation of Trem2/MerTK, galectin-3 binding assay, neuronal desialylation and toxicity assay Frontiers in cellular neuroscience Medium 35693885
2017 NEU1 interacts with perilipin 1 (Plin1) on lipid droplets under basal conditions in 3T3-L1 adipocytes, as shown by co-immunoprecipitation and co-localization. Neu1 knockdown increases glycerol release (lipolysis), Plin1 phosphorylation, and Plin1-HSL interaction upon β-adrenergic stimulation, indicating that NEU1 inhibits lipolysis by interacting with Plin1 on lipid droplets. siRNA knockdown, co-immunoprecipitation, immunofluorescent co-localization, glycerol concentration measurement, Plin1 phosphorylation analysis, Plin1-HSL interaction assay Genes to cells Medium 28429532
2022 Cardiomyocyte-specific NEU1 deficiency in mice post-myocardial infarction reduces cardiac dysfunction, hypertrophy, fibrosis, mitochondrial dysfunction, and oxidative stress. The mechanism involves NEU1 deficiency increasing SIRT1 and PGC-1α expression; SIRT1 activity inhibition or PGC-1α knockout abolished the beneficial effects of NEU1 deficiency, placing NEU1 upstream of the SIRT1/PGC-1α axis in post-MI cardiac metabolism. Cardiomyocyte-specific NEU1 KO mice (MI model), SIRT1 activity inhibitor, PGC-1α KO mice, echocardiography, Western blot, mitochondrial metabolic assays, oxidative stress assays Frontiers in cardiovascular medicine Medium 35548408
2023 NEU1 regulates coronavirus replication by controlling sialylation on the coronavirus nucleocapsid protein. Coronavirus nucleocapsid proteins from COVID-19 patients and HCoV-OC43-infected cells are heavily sialylated, and this sialylation controls RNA-binding activity and viral replication. NEU1 overexpression increased HCoV-OC43 replication, while NEU1 knockdown reduced it. A NEU1 inhibitor (Neu5Ac2en-OAcOMe) dramatically reduced HCoV-OC43 and SARS-CoV-2 replication in vitro and rescued mice from HCoV-OC43 infection-induced death. NEU1 overexpression and knockdown, NEU1 inhibitor treatment, nucleocapsid protein sialylation analysis, RNA-binding assay, in vivo mouse infection model, in vitro SARS-CoV-2 replication assay iScience Medium 36714013
2021 NEU1 associates with and desialylates CD31 only when CD31 ectodomains are homophilically engaged in postconfluent endothelial cells. Prior NEU1 silencing completely protected against CD31 desialylation in postconfluent cells. NEU1 sialidase activity is required for inhibiting tube formation, while catalytically dead NEU1-G68V had no effect. siRNA knockdown, adenoviral overexpression of WT and catalytic dead mutant G68V, lectin blotting (SNA/PNA), tube formation assay, wound migration assay The Journal of biological chemistry Medium 24550400
2009 A regulatory mutation (-519G→A) in the Neu1 promoter generates a consensus binding site for Nkx3 family repressors. Recombinant Nkx3.2 binds strongly to and preferentially represses the mutant promoter. This tissue-specific epigenetic mechanism causes reduced Neu1 expression in SM/J mice, contributing to impaired immune response and altered leukocyte adhesion/rolling. Reporter assays, recombinant Nkx3.2 binding and repression assays, intravital hepatic microcirculation imaging, SM/J mouse model Molecular genetics and metabolism Medium 19217813
2022 NEU1 haploinsufficiency drives a model of pleomorphic rhabdomyosarcoma through increased lysosomal exocytosis downstream of reduced NEU1. This is evidenced by redistribution of LAMP1 to the plasma membrane of tumor and stromal cells, establishing NEU1's negative regulation of lysosomal exocytosis as a tumor-suppressor mechanism in muscle. NEU1 haploinsufficient mouse model (Ptch1+/-/ETV7TG+/-), LAMP1 immunolocalization, single-cell analysis of exocytic cells Communications biology Medium 36127469
2023 NEU1 desialylates ATG5, an autophagy protein, under hypoxic conditions. Desialylation of ATG5 by NEU1 enhances ATG5 protein stability (via altered charge effects), promotes formation of the ATG5-ATG12-ATG16L complex, and increases autophagosome formation. NEU1 was identified as a direct interacting protein of ATG5 by co-immunoprecipitation. NEU1 knockdown or inhibition reversed hypoxia-induced autophagy and inflammatory responses in macrophages. Co-immunoprecipitation (NEU1-ATG5 interaction), NEU1 knockdown/inhibition, ATG5 sialylation analysis, autophagosome formation assay, ATG5-ATG12-ATG16L complex analysis Cellular signalling Medium 37844713
2021 NEU1 interacts with and desialylates β2 integrin in human monocytes and desialylates ICAM-1 in endothelial cells, downstream of EDP binding to the elastin receptor complex. These desialylation events are associated with increased monocyte adhesion to and transendothelial migration across endothelial cells. Proteomic validation, co-immunoprecipitation, sialidase activity assay, monocyte adhesion assay, transendothelial migration assay Cell & bioscience Medium 34903296
2018 NEU1 exists on the surface of mouse thymocytes and CD5 is a natural substrate for this cell-surface NEU1. The activity was identified by PNA-blot analysis of anti-CD5 immunoprecipitate and inhibited by the NEU1-selective inhibitor C9-butyl-amide-DANA. PNA-blot of anti-CD5 immunoprecipitate, NEU1-selective inhibitor (C9-BA-DANA), SM/J (Neu1-deficient) mouse comparison, real-time PCR Glycobiology Medium 29897583
2017 GPCR agonists (bombesin, bradykinin, angiotensin I and II) activate Neu1 sialidase and insulin receptor (IR) signaling independently of insulin, via a biased GPCR signaling platform involving neuromedin B receptor (NMBR)-MMP9-Neu1 cross-talk. The angiotensin II receptor (type I) forms a multimeric complex with Neu1, IRβ, and NMBR in both naïve and stimulated HTC-IR cells. Co-immunoprecipitation, live-cell sialidase assay, IR phosphorylation analysis, NMBR inhibitor (BIM-23127), oseltamivir phosphate (NEU1 inhibitor) Cellular signalling Medium 29277445
2016 NEU1 overexpression in pulmonary fibroblasts provokes increased collagen types I and III, accelerated MMP-14 degradation, and global gene expression changes. In lung microvascular endothelial cells, NEU1 overexpression increases T cell adhesion and disrupts capillary-like tube formation. NEU1 adenoviral delivery to mice induces lymphocyte (predominantly CD8+ T cell) accumulation in bronchoalveolar lavage and lung, plus pulmonary TGF-β and collagen elevation, recapitulating key features of lung fibrosis. Adenoviral NEU1 gene delivery (in vitro and in vivo), collagen assay, MMP-14 analysis, wounding assay, T cell adhesion assay, tube formation assay, BAL cell counting American journal of physiology. Lung cellular and molecular physiology Medium 26993524
2025 Epididymal epithelial cells secrete and transfer NEU1 onto the sperm surface, where it regulates α-2,6 sialylation to influence sperm maturation, energy metabolism, and capacitation. Inhibition of NEU1 activity markedly reduced sperm motility and fertilization capacity. NEU1 expression was lower in asthenozoospermic individuals and correlated with sperm kinematic parameters. Immunofluorescence, Western blot, flow cytometry, NEU1 activity inhibition, IVF assay, murine transfer model, sialylation analysis Cellular and molecular life sciences Medium 41329355
2024 In the periosteum, fibroblast-derived NEU1 desialylates α2,3-linked sialic acid residues on osteoclasts, disrupting cell-cell recognition and maintaining osteoclasts in a mononuclear state, thereby maintaining low cortical bone metabolic activity. Targeted NEU1 inhibition in mouse models accelerated fracture healing by up to 30%. Identified by single-cell RNA sequencing of human periosteum combined with in vitro and in vivo NEU1 inhibition experiments. Single-cell RNA sequencing, NEU1 inhibition (in vitro and in vivo), osteoclast mononuclear state analysis, fracture healing mouse model, bone quality/mechanical strength assessment Theranostics Medium 41356197

Source papers

Stage 0 corpus · 100 papers · ranked by NIH iCite citations
Year Title Journal Citations PMID
2009 Neu1 desialylation of sialyl alpha-2,3-linked beta-galactosyl residues of TOLL-like receptor 4 is essential for receptor activation and cellular signaling. Cellular signalling 160 19796680
2009 Contribution of sialidase NEU1 to suppression of metastasis of human colon cancer cells through desialylation of integrin beta4. Oncogene 153 19151752
2014 Broad and direct interaction between TLR and Siglec families of pattern recognition receptors and its regulation by Neu1. eLife 124 25187624
2013 Lysosomal NEU1 deficiency affects amyloid precursor protein levels and amyloid-β secretion via deregulated lysosomal exocytosis. Nature communications 123 24225533
2006 Monocyte differentiation up-regulates the expression of the lysosomal sialidase, Neu1, and triggers its targeting to the plasma membrane via major histocompatibility complex class II-positive compartments. The Journal of biological chemistry 121 16835219
2003 Molecular pathology of NEU1 gene in sialidosis. Human mutation 113 14517945
2009 Dependence of pathogen molecule-induced toll-like receptor activation and cell function on Neu1 sialidase. Glycoconjugate journal 106 19430901
2007 The elastin receptor complex transduces signals through the catalytic activity of its Neu-1 subunit. The Journal of biological chemistry 106 17327233
2013 Elastin-derived peptides potentiate atherosclerosis through the immune Neu1-PI3Kγ pathway. Cardiovascular research 90 24357053
2012 NEU1 sialidase expressed in human airway epithelia regulates epidermal growth factor receptor (EGFR) and MUC1 protein signaling. The Journal of biological chemistry 76 22247545
2011 Neu1 sialidase and matrix metalloproteinase-9 cross-talk is essential for Toll-like receptor activation and cellular signaling. The Journal of biological chemistry 76 21873432
2018 A positive feedback loop between IL-1β, LPS and NEU1 may promote atherosclerosis by enhancing a pro-inflammatory state in monocytes and macrophages. Vascular pharmacology 74 29371126
2009 Heterodimerization of the sialidase NEU1 with the chaperone protective protein/cathepsin A prevents its premature oligomerization. The Journal of biological chemistry 66 19666471
2014 NEU1 sialidase regulates the sialylation state of CD31 and disrupts CD31-driven capillary-like tube formation in human lung microvascular endothelia. The Journal of biological chemistry 60 24550400
2010 A crucial role of sialidase Neu1 in hyaluronan receptor function of CD44 in T helper type 2-mediated airway inflammation of murine acute asthmatic model. Clinical and experimental immunology 60 20491786
2012 NEU1 and NEU3 sialidase activity expressed in human lung microvascular endothelia: NEU1 restrains endothelial cell migration, whereas NEU3 does not. The Journal of biological chemistry 58 22403397
1998 A point mutation in the neu-1 locus causes the neuraminidase defect in the SM/J mouse. Human molecular genetics 58 9425240
2014 Therapeutic targeting of Neu1 sialidase with oseltamivir phosphate (Tamiflu®) disables cancer cell survival in human pancreatic cancer with acquired chemoresistance. OncoTargets and therapy 57 24470763
2010 Neu1 sialidase and matrix metalloproteinase-9 cross-talk is essential for neurotrophin activation of Trk receptors and cellular signaling. Cellular signalling 57 20347965
2006 Homology modeling of human sialidase enzymes NEU1, NEU3 and NEU4 based on the crystal structure of NEU2: hints for the design of selective NEU3 inhibitors. Journal of molecular graphics & modelling 56 16427342
1982 Evidence for placing the Neu-1 locus within the mouse H-2 complex. Journal of immunology (Baltimore, Md. : 1950) 56 7119440
2013 Neu1 sialidase and matrix metalloproteinase-9 cross-talk regulates nucleic acid-induced endosomal TOLL-like receptor-7 and -9 activation, cellular signaling and pro-inflammatory responses. Cellular signalling 50 23827939
2020 Sialidase NEU1 suppresses progression of human bladder cancer cells by inhibiting fibronectin-integrin α5β1 interaction and Akt signaling pathway. Cell communication and signaling : CCS 48 32164705
2012 G-protein coupled receptor agonists mediate Neu1 sialidase and matrix metalloproteinase-9 cross-talk to induce transactivation of TOLL-like receptors and cellular signaling. Cellular signalling 46 22759791
2018 Identification of CD36 as a new interaction partner of membrane NEU1: potential implication in the pro-atherogenic effects of the elastin receptor complex. Cellular and molecular life sciences : CMLS 44 30498996
2014 Expanding sialidosis spectrum by genome-wide screening: NEU1 mutations in adult-onset myoclonus. Neurology 43 24808020
2016 The NEU1-selective sialidase inhibitor, C9-butyl-amide-DANA, blocks sialidase activity and NEU1-mediated bioactivities in human lung in vitro and murine lung in vivo. Glycobiology 40 27226251
2022 NEU1 Regulates Mitochondrial Energy Metabolism and Oxidative Stress Post-myocardial Infarction in Mice via the SIRT1/PGC-1 Alpha Axis. Frontiers in cardiovascular medicine 39 35548408
2017 Biased G protein-coupled receptor agonism mediates Neu1 sialidase and matrix metalloproteinase-9 crosstalk to induce transactivation of insulin receptor signaling. Cellular signalling 39 29277445
2016 MicroRNA-125b promotes invasion and metastasis of gastric cancer by targeting STARD13 and NEU1. Tumour biology : the journal of the International Society for Oncodevelopmental Biology and Medicine 39 27220320
2016 Elevated expression of NEU1 sialidase in idiopathic pulmonary fibrosis provokes pulmonary collagen deposition, lymphocytosis, and fibrosis. American journal of physiology. Lung cellular and molecular physiology 37 26993524
2019 Modulation of TLR4 Sialylation Mediated by a Sialidase Neu1 and Impairment of Its Signaling in Leishmania donovani Infected Macrophages. Frontiers in immunology 34 31649671
2010 Detection of Neu1 sialidase activity in regulating Toll-like receptor activation. Journal of visualized experiments : JoVE 33 20864924
2015 Effects of sialidase NEU1 siRNA on proliferation, apoptosis, and invasion in human ovarian cancer. Molecular and cellular biochemistry 32 26463994
2004 Five novel mutations in the lysosomal sialidase gene (NEU1) in type II sialidosis patients and assessment of their impact on enzyme activity and intracellular targeting using adenovirus-mediated expression. Human mutation 30 14695530
2015 NEU1 Sialidase Regulates Membrane-tethered Mucin (MUC1) Ectodomain Adhesiveness for Pseudomonas aeruginosa and Decoy Receptor Release. The Journal of biological chemistry 29 25963144
2022 Neu1 Is Released From Activated Microglia, Stimulating Microglial Phagocytosis and Sensitizing Neurons to Glutamate. Frontiers in cellular neuroscience 27 35693885
2023 Structure of the immunoregulatory sialidase NEU1. Science advances 25 37205763
2013 Clinical and serial MRI findings of a sialidosis type I patient with a novel missense mutation in the NEU1 gene. Internal medicine (Tokyo, Japan) 25 23291686
2024 Neuraminidase-1 (NEU1): Biological Roles and Therapeutic Relevance in Human Disease. Current issues in molecular biology 20 39194692
2021 Interplay Between Sialic Acids, Siglec-E, and Neu1 Regulates MyD88- and TRIF-Dependent Pathways for TLR4-Activation During Leishmania donovani Infection. Frontiers in immunology 20 33763070
2019 Type 1 Sialidosis Patient With a Novel Deletion Mutation in the NEU1 Gene: Case Report and Literature Review. Cerebellum (London, England) 20 30635863
2015 Biochemical and molecular characterization of novel mutations in GLB1 and NEU1 in patient cells with lysosomal storage disorders. Biochemical and biophysical research communications 20 25600812
2022 Selective histone methyltransferase G9a inhibition reduces metastatic development of Ewing sarcoma through the epigenetic regulation of NEU1. Oncogene 19 35354905
2020 The role of Neu1 in the protective effect of dipsacoside B on acetaminophen-induced liver injury. Annals of translational medicine 19 32793668
2023 Ameliorative effects of the Coptis inflorescence extract against lung injury in diabetic mice by regulating AMPK/NEU1 signaling. Phytomedicine : international journal of phytotherapy and phytopharmacology 18 37516057
2021 Acacetin ameliorates insulin resistance in obesity mice through regulating Treg/Th17 balance via MiR-23b-3p/NEU1 Axis. BMC endocrine disorders 18 33781239
2013 Identification of lysosomal sialidase NEU1 and plasma membrane sialidase NEU3 in human erythrocytes. Journal of cellular biochemistry 18 22903576
2005 Overexpression of MyoD-inducible lysosomal sialidase (neu1) inhibits myogenesis in C2C12 cells. Experimental cell research 18 16216242
2021 Potential Role of NEU1 in Hepatocellular Carcinoma: A Study Based on Comprehensive Bioinformatical Analysis. Frontiers in molecular biosciences 17 34513919
2002 Identification of a CTL4/Neu1 fusion transcript in a sialidosis patient. FEBS letters 17 12067718
2019 Clinical and electrophysiological characteristics of a type 1 sialidosis patient with a novel deletion mutation in NEU1 gene. Journal of the Formosan Medical Association = Taiwan yi zhi 16 31371146
2021 Therapeutic Potential of Neu1 in Alzheimer's Disease Via the Immune System. American journal of Alzheimer's disease and other dementias 15 33719595
2019 Long non-coding RNA TOB1-AS1 modulates cell proliferation, apoptosis, migration and invasion through miR-23a/NEU1 axis via Wnt/b-catenin pathway in gastric cancer. European review for medical and pharmacological sciences 15 31799657
2019 MicroRNA-205 ameliorates lipid accumulation in non-alcoholic fatty liver disease through targeting NEU1. European review for medical and pharmacological sciences 15 31799678
2015 Lysosomal localization of Japanese medaka (Oryzias latipes) Neu1 sialidase and its highly conserved enzymatic profiles with human. Gene 15 26432003
1986 Electrophoretic analysis of liver neuraminidase-1 variation in mice and additional evidence concerning the location of NEU-1. Journal of immunogenetics 13 3745925
2020 Establishment and characterization of Neu1-knockout zebrafish and its abnormal clinical phenotypes. The Biochemical journal 12 32686823
2017 Sialidosis Type 1 with a Novel Mutation in the Neuraminidase-1 (NEU1) Gene. Indian journal of pediatrics 12 28138907
2015 Autophagy is decreased in triple-negative breast carcinoma involving likely the MUC1-EGFR-NEU1 signalling pathway. International journal of clinical and experimental pathology 12 26191126
2023 Targeting intracellular Neu1 for coronavirus infection treatment. iScience 11 36714013
2022 NEU1-A Unique Therapeutic Target for Alzheimer's Disease. Frontiers in pharmacology 11 35847014
2009 A point mutation in the neu1 promoter recruits an ectopic repressor, Nkx3.2 and results in a mouse model of sialidase deficiency. Molecular genetics and metabolism 11 19217813
2023 Desialylation of ATG5 by sialidase (NEU1) promotes macrophages autophagy and exacerbates inflammation under hypoxia. Cellular signalling 10 37844713
2021 NEU1 is more abundant in uveitic retina with concomitant desialylation of retinal cells. Glycobiology 10 33677598
2021 The role of neuraminidase 1 (NEU1) in cytokine release by primary mouse mesangial cells and disease outcomes in murine lupus nephritis. Autoimmunity 10 33749450
2021 Characterization of novel interactions with membrane NEU1 highlights new regulatory functions for the Elastin Receptor Complex in monocyte interaction with endothelial cells. Cell & bioscience 10 34903296
2018 Parental Whole-Exome Sequencing Enables Sialidosis Type II Diagnosis due to an NEU1 Missense Mutation as an Underlying Cause of Nephrotic Syndrome in the Child. Kidney international reports 10 30450471
2017 Neu1 sialidase interacts with perilipin 1 on lipid droplets and inhibits lipolysis in 3T3-L1 adipocytes. Genes to cells : devoted to molecular & cellular mechanisms 10 28429532
2024 The role of sialidase Neu1 in respiratory diseases. Respiratory research 9 38500102
2021 The sialidase NEU1 directly interacts with the juxtamembranous segment of the cytoplasmic domain of mucin-1 to inhibit downstream PI3K-Akt signaling. The Journal of biological chemistry 9 34688655
2020 Novel Nile tilapia Neu1 sialidases: Molecular cloning and biochemical characterization of the sialidases Neu1a and Neu1b. Gene 9 32184168
2016 Multigene panel next generation sequencing in a patient with cherry red macular spot: Identification of two novel mutations in NEU1 gene causing sialidosis type I associated with mild to unspecific biochemical and enzymatic findings. Molecular genetics and metabolism reports 9 27942463
2025 Genetic Insights and Clinical Implications of NEU1 Mutations in Sialidosis. Genes 8 40004480
2022 Haploinsufficiency of the lysosomal sialidase NEU1 results in a model of pleomorphic rhabdomyosarcoma in mice. Communications biology 8 36127469
2021 Discovery of NEU1 as a candidatedone. renal biomarker for proliferative lupus nephritis chronicity. Lupus science & medicine 8 34872988
2022 Alteration of the neuronal and glial cell profiles in Neu1-deficient zebrafish. Glycoconjugate journal 7 35877057
2017 Suppression of Neu1 sialidase delays the absorption of yolk sac in medaka (Oryzias latipes) accompanied with the accumulation of α2-3 sialo-glycoproteins. Biochimie 7 28111290
2023 Lysosomal sialidase NEU1, its intracellular properties, deficiency, and use as a therapeutic agent. Glycoconjugate journal 6 38147151
2022 NEU1 and NEU3 enzymes alter CD22 organization on B cells. Biophysical reports 6 36425332
2014 In silico identification of new putative pathogenic variants in the NEU1 sialidase gene affecting enzyme function and subcellular localization. PloS one 6 25153125
2024 Neu1 deficiency and fibrotic lymph node microenvironment lead to imbalance in M1/M2 macrophage polarization. Frontiers in immunology 5 39346907
2018 Existence of NEU1 sialidase on mouse thymocytes whose natural substrate is CD5. Glycobiology 5 29897583
1986 Genetic analysis of liver neuraminidase isozymes in Rattus norvegicus: independent control of NEU-1 and NEU-2 phenotypes. Genetics 5 3770467
2025 New use of an old drug: mechanism of oseltamivir phosphate inhibiting liver cancer through regulation of lipophagy via NEU1. Frontiers in pharmacology 4 40196362
2025 Fucoidan alleviates renal fibrosis in mice via Akkermansia muciniphila-mediated suppression of NEU1-TLR4-NFκB signaling axis. Phytomedicine : international journal of phytotherapy and phytopharmacology 4 40663936
2020 Heterozygous structural variation mimicking homozygous missense mutations in NEU1 associated with presenting clinical signs in eyes alone. Ophthalmic genetics 4 32270733
2019 Identification of a homozygous deletion of the NEU1 gene in a patient with type II sialidosis presenting isolated fetal ascites and central nervous system hypoplasia. Hippokratia 4 32742167
2018 Generation of novel induced pluripotent stem cell (iPSC) line from a 16-year-old sialidosis patient with NEU-1 gene mutation. Stem cell research 4 29414417
2017 As human lung microvascular endothelia achieve confluence, src family kinases are activated, and tyrosine-phosphorylated p120 catenin physically couples NEU1 sialidase to CD31. Cellular signalling 4 28343945
2025 Inhibition of class IIa HDACs reduces neuroinflammation via NEU1-LAMP1 regulation and promotes M2 macrophage polarization in ischemic stroke. Brain research 3 40581210
2022 Correlation Between Sialidase NEU1 mRNA Expression Changes in Autism Spectrum Disorder. Frontiers in psychiatry 3 35757207
2025 Sotorasib-impaired degradation of NEU1 contributes to cardiac injury by inhibiting AKT signaling. Cell death discovery 2 40221400
2025 NEU1-Mediated Extracellular Vesicle Glycosylation in Alzheimer's Disease: Mechanistic Insights into Intercellular Communication and Therapeutic Targeting. Pharmaceuticals (Basel, Switzerland) 2 40573316
2024 Stenotrophomonas maltophilia provokes NEU1-mediated release of a flagellin-binding decoy receptor that protects against lethal infection. iScience 2 39314239
2021 Clinical Exome Sequencing Enables Congenital Sialidosis Type II Diagnosis in Two Siblings Presenting with Unreported Clinical Features from a Rare Homozygous Sequence Variant p.(Tyr370Cys) in NEU1. Molecular syndromology 2 34421504
2026 Fibroblast-derived NEU1 as a therapeutic target for improving cortical bone integrity and fracture healing. Theranostics 1 41356197
2025 Epididymal epithelial cells facilitate NEU1 loading to modulate sperm α-2,6 sialylation, enhance maturation and motility. Cellular and molecular life sciences : CMLS 1 41329355
2024 HDAC1-Mediated Downregulation of NEU1 Exacerbates the Aggressiveness of Cervical Cancer. Critical reviews in eukaryotic gene expression 1 38505872
2024 H1N1 swine influenza viruses upregulate NEU1 expression through histone H3 acetylation regulated by HDAC2. Virology 1 39566266

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