Selected-GenAtlas references SOURCE GeneCards NCBI Gene Swiss-Prot Orphanet Ensembl
HGNC UniGene Nucleotide OMIM UCSC
Home Page
FLASH GENE
Symbol SPIB contributors: mct - updated : 19-11-2010
HGNC name Spi-B transcription factor (Spi-1/PU.1 related)
HGNC id 11242
PROTEIN
PHYSICAL PROPERTIES
STRUCTURE
motifs/domains
  • a conserved ETS domain mediating specific DNA binding
  • a weakly acidic N-terminal transactivation domain (TAD)
  • a second TAD rich in proline, serine and threonine
    • HOMOLOGY
    interspecies ortholog to murine Spib
    homolog to C elegans C18E3.9
    intraspecies homolog to Spi-1/PU.1 putative oncogene
    Homologene
    FAMILY
  • SPI-group ETS-protein transcription factors family
    • CATEGORY transcription factor
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm,cytoskeleton,microtubule
    intracellular,nucleus
    basic FUNCTION
  • regulating transcription from Pol II promoter
  • affecting SPIB function by recruiting factors involved in SPIB activity
  • key regulator of plasmacytoid dendritic cells development, whereas T-cell development is critically dependent on GATA3
  • implicated in signaling pathways downstream of the B cell receptor and is essential for germinal center formation and maintenance
  • playing a role in maintenance of germinal center and memory B cells by direct repression of major plasma cell factors and thereby plasma cell differentiation
  • may be contributes to plasmacytoid dendritic cells development by promoting TCF4 activity
  • important mediator of both B-cell receptor signaling and early T-cell lineage decisions
  • also induces the development of plasmacytoid dendritic cells and can mediate IL7R, REL and IFNG–induced CD40 expression
  • as specific transcription factors SPI1, and SPIC, regulate follicular B cell differentiation
  • SPI1 and SPIB are essential transcriptional regulators of B-cell differentiation as well as novel tumor suppressors in the B-cell lineage
    • CELLULAR PROCESS nucleotide, transcription, regulation
    PHYSIOLOGICAL PROCESS
    text transcription activator
    PATHWAY
    metabolism
    signaling
    a component
    INTERACTION
    DNA binding to a purine-rich sequence the PU box (5'-GAGGAA-3') acting as a
    lymphoid-specific enhancer
    RNA
    small molecule
    protein
  • interacting with IRF4 and with CREBBP, EP300, SPI1-related, JUN and TBP
  • direct target gene critically regulated by POU2AF1
    • cell & other
    REGULATION
    ASSOCIATED DISORDERS
    corresponding disease(s)
    Susceptibility to primary biliary cirrhosis
    Variant & Polymorphism SNP
  • rs3745516 within SPIB strongly associated with primary biliary cirrhosis
    • Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS
  • mice deficient in both Spib and Spi1 in the B-cell lineage had reduced frequencies of B cells as well as impaired B-cell differentiation, and developed pre-B cell acute lymphoblastic leukemia before 30 weeks of age