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FLASH GENE
Symbol IKZF3 contributors: mct - updated : 19-11-2015
HGNC name IKAROS family zinc finger 3 (Aiolos)
HGNC id 13178
Location 17q12      Physical location : 37.921.200 - 38.020.441
Synonym name
  • zinc finger protein, subfamily 1A, 3 (Aiolos)
  • zinc finger protein Aiolos
  • Synonym symbol(s) AIOLOS, ZNF1A3, EVI112, ZNFN1A3, AIO
    DNA
    TYPE functioning gene
    STRUCTURE 106.47 kb     8 Exon(s)
    regulatory sequence Promoter
    Binding site   transcription factor
    text structure
  • a positive regulatory region important for the promoter activity of the Aiolos gene
  • within this positive regulatory region, NFKB, Ikaros and AP4-binding sites are critical for Aiolos promoter activity
  • MAPPING cloned Y linked N status confirmed
    RNA
    TRANSCRIPTS type messenger
    identificationnb exonstypebpproduct
    ProteinkDaAAspecific expressionYearPubmed
    8 splicing 9686 57.9 509 - 2001 11745366
    7 splicing 9518 51.5 453 - 2001 11745366
  • lacking an alternate in-frame exon, compared to variant 1
  • 7 splicing 9659 53.3 470 - 2001 11745366
  • lacking an alternate in-frame exon, compared to variant 1
  • 7 splicing 9569 53.6 470 - 2001 11745366
  • lacking an alternate in-frame exon, compared to variant 1
  • 6 splicing 9401 46.9 414 - 2001 11745366
  • lacking two alternate in-frame exons, compared to variant 1
  • 6 splicing 9452 49 431 - 2001 11745366
  • lacking two alternate in-frame exons, compared to variant 1
  • 6 - 9467 - 436 - 2001 11745366
    7 - 9584 - 475 - 2001 11745366
    4 - 9023 - 288 - 2001 11745366
    5 - 9140 - 327 - 2001 11745366
    6 - 9257 - 366 - 2001 11745366
    7 - 9425 - 422 - 2001 11745366
    5 - 9284 - 375 - 2001 11745366
    EXPRESSION
    Type
       expressed in (based on citations)
    organ(s)
    SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
    Digestiveintestinesmall intestine  lowly
     liver   lowly
     pharynx   predominantly
    Lymphoid/Immunespleen    
     thymus   highly
    Respiratorylung   lowly
    tissue
    SystemTissueTissue level 1Tissue level 2LevelPubmedSpeciesStageRna symbol
    Blood / hematopoietic     Homo sapiens
    Lymphoid    
    cells
    SystemCellPubmedSpeciesStageRna symbol
    Blood/Hematopoieticleukocyte Homo sapiens
    Lymphoid/ImmuneB cell Homo sapiens
    cell lineage
    cell lines
    fluid/secretion highly in lymph, moderately in blood
    at STAGE
    PROTEIN
    PHYSICAL PROPERTIES
    STRUCTURE
    motifs/domains
  • four N terminal C2H2-type zinc fingers comprising the DNA binding domain
  • two C terminal C2H2-type zinc fingers mediating protein dimerization
  • conjugated PhosphoP , MetalloP
    HOMOLOGY
    interspecies homolog to murine Zfpn1a3 (87 pc)
    homolog to rattus Zfpn1a3 (87.9 pc)
    intraspecies homolog to Ikaros,Aiolos
    Homologene
    FAMILY
  • IKAROS family of C2H2-type zinc-finger proteins
  • zinc finger protein, subfamily 1A
  • CATEGORY transcription factor
    SUBCELLULAR LOCALIZATION     plasma membrane
        intracellular
    intracellular,cytoplasm
    intracellular,nucleus,chromatin/chromosome
    basic FUNCTION
  • playing an antiproliferative role in the lymphocyte differentiation
  • playing a role in the control of T cell death by regulating the expression of BCL2
  • novel role for POU2AF1 anf IKZF3 in controlling the transcription and nuclear organization of genes involved in pre-BCR function
  • plays a crucial role in the control of lymphocyte differentiation and proliferation
  • Ikaros family members are critical for a functional immune system that confers protection against invading pathogens, while minimising the risk of leukaemic transformation, immune pathology, and autoimmunity
  • plays an important role in the control of mature B lymphocyte differentiation and maturation
  • involved in cell survival by regulating the expression of some BCL2 family members
  • promotes TH17 differentiation by directly silencing IL2 expression
  • CELLULAR PROCESS cell life, differentiation
    nucleotide, chromatin organization, remodeling
    nucleotide, transcription, regulation
    PHYSIOLOGICAL PROCESS development
    text mesoderm development
    PATHWAY
    metabolism
    signaling
    a component
  • homo or heterodimerizing with either other member of the family
  • INTERACTION
    DNA binding
    RNA
    small molecule metal binding,
  • Zn2+
  • protein
  • IKZF4
  • IKZF5
  • IKZF3 and IKZF1 bind to the MYC promoter and directly suppress MYC expression in pre-B cells
  • downregulation of MYC by IKZF1 and IKZF3 is necessary for the induction of CDKN1B and downregulation of CCND3
  • STAT3 and the Ikaros zinc finger transcription factors IKZF3, IKZF1 cooperate to regulate BCL66 expression
  • cell & other
    REGULATION
    Other transcription factors Ikaros and NFKB involved in the control of Aiolos expression
    ASSOCIATED DISORDERS
    corresponding disease(s)
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    tumoral   deletion    
    in acute lymphoblastic leukaemia
    tumoral     --over  
    in B-Chronic lymphocytic leukemia
    Susceptibility to systemic lupus erythematosus
    Variant & Polymorphism SNP
  • rs8076347, rs9913957, rs8079075, associated to systemic lupus erythematosus
  • Candidate gene
    Marker
  • IKZF3 deregulation by epigenetic modifications may be a hallmark of Chronic lymphocytic leukemia
  • Therapy target
    ANIMAL & CELL MODELS