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FLASH GENE
Symbol ZNF521 contributors: mct - updated : 18-11-2009
HGNC name zinc finger protein 521
HGNC id 24605
Location 18q11.2      Physical location : 22.641.887 - 22.932.214
Synonym name
  • LYST-interacting protein 3
  • early hematopoietic zinc finger
  • Synonym symbol(s) EHZF, Evi3, MGC142182, MGC142208, DKFZp564D0764
    DNA
    TYPE functioning gene
    STRUCTURE 290.33 kb     8 Exon(s)
    MAPPING cloned Y linked N status provisional
    RNA
    TRANSCRIPTS type messenger
    identificationnb exonstypebpproduct
    ProteinkDaAAspecific expressionYearPubmed
    8 - 4985 - 1131 - 2009 19049973
    EXPRESSION
    Type widely
       expressed in (based on citations)
    organ(s)
    SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
    Cardiovascularvessel     Homo sapiens
    Endocrineneuroendocrinepituitary  highly Homo sapiens
    Hearing/Equilibriumearinnercochlea highly Homo sapiens
    Nervousbrainhindbraincerebellum highly Homo sapiens
    Reproductivefemale systemplacenta    Homo sapiens
    cells
    SystemCellPubmedSpeciesStageRna symbol
    Blood/Hematopoieticprogenitor cell Homo sapiens
    cell lineage CD34+ progenitor, high expression in the most immature cells of the haematopoietic system and declines with differentiation
    cell lines
    fluid/secretion
    at STAGE
    physiological period embryo, pregnancy
    Text
  • placenta
  • expressed in embryonic stem
  • cells, osteoblasts, chondrocytes and in subsets
    of neurons
    PROTEIN
    PHYSICAL PROPERTIES
    STRUCTURE
    motifs/domains
  • an N-terminal transcriptional repressor motif with 13-AA motif, that binds to components of the nuclear remodelling and histone deacetylation (NuRD) complex and is conserved in several trascriptional co-repressors
  • 30 zinc finger domains
  • HOMOLOGY
    interspecies ortholog to murine Zfp521
    homolog to murine Evi3
    Homologene
    FAMILY
  • krueppel C2H2-type zinc-finger protein family
  • CATEGORY regulatory
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,nucleus
    basic FUNCTION
  • inhibiting the transcriptional activity of early B-cell factor (EBF1), and regulating EBF1 activity in B-cell leukemia
  • playing a relevant role in the control of hematopoiesis and might be implicated in the development of hematopoietic malignancies
  • inhibitor of the transcription factor EBF1, implicated in the differentiation of neural progenitors and in the specification of the B-cell lineage
  • having a a possible role in development, stem cell regulation and oncogenesis
  • relevant player in the regulation of the homeostasis of the hematopoietic stem/progenitor cell compartment
  • plays an important role in the control of B-cell development by inhibiting the activity of EBF1, a master factor in B-lineage specification)
  • can antagonize B-cell development and may potentially contribute to conserve the multipotency of primitive lympho-myeloid progenitors by preventing or delaying their EBF1-driven commitment toward the B-cell lineage
  • could modulate B-cell development by suppressing
  • EBF1 activity and its molecular mechanism of action
  • appears to function as a transcriptional coactivator
  • together with EP300, adding to the functional
    diversity of ZNF521
  • is the intrinsic repressor of ZNF423 and hence of adipocyte commitment during BMP-induced mesenchymal precursor differentiation
  • CELLULAR PROCESS nucleotide, transcription, regulation
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism
    signaling
    member of the BMP signaling pathway
    a component
    INTERACTION
    DNA
    RNA
    small molecule metal binding,
  • Zn2+
  • protein
  • interacting with EBF1 (misexpression of ZNF521 initiating tumorigenesis by perturbing B-cell development via an interaction with EBF1)
  • direct interaction of EHZF with GATA1 was demonstrated(EHZF modulates erythroid cell differentiation through direct binding with GATA1)
  • interacting with RUNX2 (the balance and molecular interplay between ZNF521 and RUNX2 contribute to the control of osteoblast differentiation, skeletal development, and bone homeostasis)
  • suppresses EBF1 activity via its C-terminal
  • zinc fingers
    cell & other
    REGULATION
    ASSOCIATED DISORDERS
    corresponding disease(s)
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    tumoral     --over  
    acute myelogenous leukemia
    tumoral     --over  
    in CD34+ progenitors and hematopoietic malignancies
    tumoral     --over  
    in most acute myelogenous leukaemias and is particularly high in those with rearrangements of the MLL gene, where EHZF may contribute to the leukaemic phenotype
    Susceptibility
    Variant & Polymorphism
    Candidate gene
  • is a cooperative gene for TCF3-HLF to develop acute B-lineage leukemia
  • Marker
    Therapy target
    ANIMAL & CELL MODELS