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FLASH GENE
Symbol CNPY3 contributors: mct/npt - updated : 09-02-2016
HGNC name canopy 3 homolog (zebrafish)
HGNC id 11968
EXPRESSION
Type
   expressed in (based on citations)
organ(s)
SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
Digestiveintestinelarge intestinecolon  
Nervousbrain    
Respiratorylung    
cells
SystemCellPubmedSpeciesStageRna symbol
Skin/Tegumentkeratinocyte
cell lineage
cell lines
fluid/secretion
at STAGE
PROTEIN
PHYSICAL PROPERTIES
STRUCTURE
motifs/domains
  • N-terminal signal sequence
  • a domain of unknown function (DUF3456),
  • a putative C-terminal ER retention signal
  • HOMOLOGY
    interspecies homolog to rattus LOC316225
    Homologene
    FAMILY
  • canopy family
  • CATEGORY chaperone/stress , immunity/defense
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm,organelle,lumen
    intracellular,cytoplasm,organelle,endoplasmic reticulum
    text resides in the endoplasmic reticulum (Takahashi 2007)
    basic FUNCTION
  • required for multiple TLR responses (Takahashi 2007)
  • regulates the subcellular distribution and response of multiple TLRs and is required for both innate and adaptive immune responses (Takahashi 2007)
  • chaperone-like endoplasmic reticulum (ER)-resident protein required for the proper subcellular distribution of multiple TLRs (Kiyokawa 2008)
  • with HSP90B1, differentially regulate TLR4 translocation to the cell surface (Saitoh 20009)
  • with UNC93B1, and HSP90B21, play a role in TLR localisation (McGettrick 2010)
  • CNPY3 is a TLR-specific cochaperone for HSP90B1
  • distinct roles for CNPY3 and CNPY4 in the regulation of TLR1 surface expression
  • TLR1, TLR2 and TLR6 were expressed on the surface of B cells, monocytes and dendritic cells in a manner dependent on TLR-specific chaperone CNPY3
  • co-chaperone in the endoplasmic reticulum that regulates the subcellular distribution and responses of multiple Toll-like receptors
  • perform essential roles in brain function in addition to known Toll-like receptor-dependent immune responses
  • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism
    signaling
    a component
    INTERACTION
    DNA
    RNA
    small molecule
    protein
  • associated with TLR4, regulates cell surface expression of TLR4 and is associated with the immature form of TLR4 but not with LY96 or TLR2 (Wakabayashi 2006)
  • differentially interacts with each TLR (suggest that a single-nucleotide change in the CNPY3 gene influences not only the strength of TLR responses but can also alter the relative activity of each TLR) (Kiyokawa 2008)
  • HSP90B1 directly interacts with CNPY3, and the complex dissociates in the presence of adenosine triphosphate (ATP)
  • ER-resident TLR chaperones, CNPY3, CNPY4 act as positive and negative regulators of TLR1 surface trafficking, respectively
  • collectively, cell surface expression of TLR5 is dependent on CNPY3 and restricted to neutrophils, classical monocytes and specific dendritic cells subsets
  • cell & other
    REGULATION
    induced by IFNG1 (expression is strongly induced in human monocytes by IFNG1)
    ASSOCIATED DISORDERS
    corresponding disease(s) EIEE60
    Susceptibility to West syndrome (WS)
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS
  • Prat4a-/- bone marrow chimeric mice were resistant to lipopolysaccharide-induced sepsis (Takahashi 2007)
  • Cnpy3-knockout mice exhibited spastic or dystonic features under resting conditions and hyperactivity