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FLASH GENE
Symbol WBP11 contributors: mct/npt - updated : 07-02-2024
HGNC name WW domain binding protein 11
HGNC id 16461
EXPRESSION
Type ubiquitous
   expressed in (based on citations)
organ(s)
SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
Endocrinethyroid   highly
Nervousbrain    
Reproductivefemale systemuteruscervix highly
 male systemtestis  highly
Respiratorylung    
tissue
SystemTissueTissue level 1Tissue level 2LevelPubmedSpeciesStageRna symbol
Epithelialbarrier liningretinal pigment epithelium (RPE)  
Muscularstriatumskeletal  
cell lineage
cell lines
fluid/secretion
at STAGE
PROTEIN
PHYSICAL PROPERTIES
STRUCTURE
motifs/domains
  • nuclear localization signal in the N-terminus ,
  • two distinct PP1-binding domains involving a RVXF (Arg-Val-Xaa-Phe) motif
  • proline-rich C-terminal domain required for its subnuclear targeting to the speckles and binding PQBP1
  • mono polymer heteromer , dimer
    HOMOLOGY
    interspecies ortholog to murine Wbp11
    Homologene
    FAMILY
    CATEGORY DNA associated
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm,cytosolic
    intracellular,nucleus,nucleoplasm,nuclear bodies,nuclear speckles
    text
  • largely nuclear, but it accumulated in the cytoplasm following UV- or X-radiation
  • colocalized with mRNA splicing factors and intermediate filament containing perinuclear networks
  • basic FUNCTION
  • functioning as an mRNA factory in the nucleus
  • may function as a component of an mRNA factory in the nucleus
  • nucleocytoplasmic shuttling protein, implicated in pre-mRNA splicing, by nucleocytoplasmic shuttling
  • pre-mRNA splicing factor WBP11, is a protein ligand of PQBP1 (Nicolaescu 2008)
  • essential roles for PQBP1 and a binding partner, WBP11, in early development
  • WBP11 is required for centriole duplication and/or stability
  • WBP11 and SNW1 promote the removal of a specific subset of introns
  • CELLULAR PROCESS nucleotide, RNA splicing
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism
    signaling
    a component
  • PQBP1, WBP11 complex
  • component of the spliceosomes
  • WBP11-PQBP1-induced nuclear inclusions are distinct from the protein aggregates that are associated with polyglutamine diseases and represent dynamic nucleoplasmic heteropolymers of WBP11 and PQBP1 (Nicolaescu 2008)
  • one of the functional partners of PQBP1 from a potentially large repertoire of other signaling ligands
  • INTERACTION
    DNA binding to poly (rG) and G-rich single strand
    RNA
    small molecule
    protein
  • interacts with protein Ser/Thr phosphatase-1 (PP1) and with the polyglutamine-tract-binding protein 1 (PQBP1)
  • interacting with WBP4 (significantly, tandem WW domains of WBP4 are connected by a highly flexible region, enabling their simultaneous interaction with two proline-rich motifs of WBP11) (Huang 2009)
  • PQBP1 and WBP11 negatively modulates the U5-15kD binding of PQBP1 by an allosteric mechanism
  • WBP4 interacts with the U2-associated proteins SF3B4, WBP1
  • cell & other
    REGULATION
    ASSOCIATED DISORDERS
    corresponding disease(s) VCTERL
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    constitutional       loss of function
    LOF WBP11 and WBP4 variants should be considered as a possible cause of VACTERL association
    constitutional     --low  
    causes centriole duplication defects, in part by causing a rapid decline in the level of TUBGCP6
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS