Selected-GenAtlas references SOURCE GeneCards NCBI Gene Ensembl
HGNC UniGene Nucleotide OMIM UCSC
Home Page
FLASH GENE
Symbol ZBTB18 contributors: mct - updated : 14-01-2017
HGNC name zinc finger and BTB domain containing 18
HGNC id 13030
PROTEIN
PHYSICAL PROPERTIES
STRUCTURE
motifs/domains
  • a BTB/POZ zinc finger N terminal domain
  • four zinc finger-like domains at the C terminus
    • conjugated MetalloP
    HOMOLOGY
    interspecies homolog to murine Znf238
    Homologene
    FAMILY zinc finger protein family, C2H2 type
    CATEGORY regulatory , transcription factor
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm,cytoskeleton,microtubule
    intracellular,nucleus,nucleoplasm
    intracellular,nucleus,chromatin/chromosome
    basic FUNCTION
  • playing a role in transcriptional repression in the central nervous system
  • may play an important role in brain development and function
  • potentially controls cell division of progenitor cells and regulates the survival of postmitotic cortical neurons
  • is expressed in postmitotic brain cells and inhibits brain tumor growth
  • is involved in control of neuronal progenitor cells and survival of cortical neurons, and is implicated with CEP170 as novel genes causative for corpus callosal abnormalities in patients with a terminal 1q deletion
  • may act to favor neuronal differentiation and brain growth by coherently repressing multiple proneurogenic genes in a timely manner
  • key regulator that controls the self-renewal and differentiation of neural stem cells (NSCs) and restriction of astrogenesis by repressing ID1, ID2, ID3, ID4 genes during corticogenesis
  • is essential for the growth and organization of the cerebellum and regulates the development of both GABAergic and glutamatergic neurons
  • key regulator of neuronal migration via multipolar-to-bipolar transition
  • functions in cell-autonomous multipolar-to-bipolar transition, independent of cell-cycle exit
  • is a key player in the transcriptional control of cell migration in the developing cerebral cortex
    • CELLULAR PROCESS cell life
    nucleotide, transcription, regulation
    cell organization/biogenesis
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism
    signaling
    a component
    INTERACTION
    DNA binding
    RNA
    small molecule metal binding,
    Zn2+
    protein
  • recruiting DNMT3A to HDAC1 to silence transcription
  • NEUROG2 is a direct upstream activator of ZBTB18 expression
  • ZBTB18 represses NEUROG2 transcription to regulate the NEUROG2-RND2 pathway
  • negative regulation of RND2 by ZNF238 was important for cortical cell migration
  • CTBP2 interacted with ZBTB18 to affect biological characteristics of GBM cells, and the tumor growth, which may be related to the SHH-GLI1 pathway
    • cell & other
    REGULATION
    ASSOCIATED DISORDERS
    corresponding disease(s) DEL1Q44
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    constitutional   deletion    
    neural-specific deletion of the BTB/POZ zinc-finger transcription factor-encoding gene ZBTB18 causes severe cerebellar hypoplasia and developmental failure of Purkinje neurons, Bergmann glia and granule neurons
    tumoral       loss of function
    in glioblastoma (GBM)
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS
  • Rp58 knockout (KO) mice demonstrated enhanced astrogenesis accompanied with an excess of neural stem cells (NSCs)
  • mice with loss of Rp58 die at birth with neocortical defects