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FLASH GENE
Symbol CNPY3 contributors: mct/npt - updated : 09-02-2016
HGNC name canopy 3 homolog (zebrafish)
HGNC id 11968
DNA
TYPE functioning gene
STRUCTURE 10.15 kb     6 Exon(s)
MAPPING cloned Y linked N status provisional
RNA
TRANSCRIPTS type messenger
identificationnb exonstypebpproduct
ProteinkDaAAspecific expressionYearPubmed
6 - 1783 - 278 - 2008 18780723
7 - 1883 - 311 - 2008 18780723
5 - 1660 - 189 - 2008 18780723
4 - 1583 - 116 - 2008 18780723
3 - 1521 - 151 - 2008 18780723
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