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FLASH GENE
Symbol NEU1 contributors: mct/pgu - updated : 03-12-2012
HGNC name sialidase 1 (lysosomal sialidase)
HGNC id 7758
PROTEIN
PHYSICAL PROPERTIES
STRUCTURE
motifs/domains
  • enzyme containing the F/YRTP motif in N terminal region conserved in all sialidases and five Asp boxes (S-X-D-X-G-X-X-T/W)
  • mono polymer homomer , heteromer , dimer , oligo
    HOMOLOGY
    interspecies homolog to murine Neu1 (83.9pc)
    homolog to rattus Neu1 (83.7pc)
    Homologene
    FAMILY
  • glycosyl hydrolase 33 family, neuraminidases family
  • CATEGORY enzyme
    SUBCELLULAR LOCALIZATION     plasma membrane
        intracellular
    intracellular,cytoplasm,organelle,lumen
    intracellular,cytoplasm,organelle,membrane
    intracellular,cytoplasm,organelle,endoplasmic reticulum
    intracellular,cytoplasm,organelle,lysosome
    intracellular,nuclear envelope,ext
    text
  • located in the outer membrane of the nuclear envelope
  • localized to both the plasma membrane and the perinuclear region
  • NEU1 and NEU3 are present on erythrocyte plasma membrane
  • basic FUNCTION
  • involved in stepwise degradation of complex type of oligosaccharide structure (glycoprotein catabolism), and cleavage of sialic acid from GM1 gangliosides
  • upregulated during T cell activation
  • can desialylate neighboring microfibrillar glycoproteins and facilitate the deposition of insoluble elastin, which contributes to the maintenance of cellular quiescence
  • desialylates and functionally inactivates adjacent receptors interacting with the mitogenic growth factors PDGF-BB and IGF2
  • negatively regulates lysosomal exocytosis in hematopoietic cells by processing the sialic acids on the lysosomal membrane protein LAMP1
  • targeted to the cell surface in a multiprotein complex that facilitates elastic fiber assembly
  • having a possible regulatory role in the early stages of myogenesis
  • negative regulator of lysosomal exocytosis
  • has been shown to regulate phagocytosis in macrophage cells
  • NEU1 and MMP9 in complex with TLR receptors at the ectodomain form a molecular organizational signaling platform on the cell surface that is essential for ligand activation of TLR receptors and cellular signaling
  • novel role as an intermediate in the initial process of ligand-induced TLR receptor activation and subsequent cellular signaling
  • NEU1 restrains endothelial cell migration, whereas NEU3 does not
  • its catalytic activity may offer an additional level of regulation over the airway epithelial response to ligands, pathogens, and injurious stimuli
  • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS nervous system , endocytosis transport , ossification
    PATHWAY
    metabolism carbohydrate
    signaling
    a component
  • complexing with beta-galactosidase and protective cathepsin A (CTSA)
  • subunit of the cell surface elastin receptor
  • in absence of CTSA, self-associating into chain-like oligomers
  • INTERACTION
    DNA
    RNA
    small molecule
    protein
  • associating with the protective protein CTSA, GLB1 and GALNS
  • tightly regulates neurotrophin receptors like NTRK1 and NTRK2, which involve NEU1 and matrix metalloproteinase-9 (MMP9) cross-talk in complex with the receptors
  • associated with EGFR and MUC1, and NEU1-EGFR association was regulated by EGF stimulation
  • cell & other
  • membrane associated
  • REGULATION
    Other targeted to the lysosome by M6P receptor mediated pathway, activated and stabilized
    ASSOCIATED DISORDERS
    corresponding disease(s) NEU1D
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    constitutional     --low  
    in sperm of some male idiopathic infertility cases
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS