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FLASH GENE
Symbol EGR2 contributors: mct/npt/pgu - updated : 09-04-2015
HGNC name early growth response 2
HGNC id 3239
PROTEIN
PHYSICAL PROPERTIES
STRUCTURE
motifs/domains
  • three tandem zinc finger C2H2 type
  • DNA binding domain
  • one class 2 flanked by two class 1
  • one inhibitory domain
    • HOMOLOGY
    interspecies homolog to Drosophila Krox-20
    intraspecies homolog to Krüppel 20
    Homologene
    FAMILY
  • EGR C2H2-type zinc-finger protein family
    • CATEGORY transcription factor , tumor suppressor
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm
    intracellular,nucleus
    basic FUNCTION
  • involved in early myelination of the peripheral nervous system
  • mediator of the PTEN growth suppressive signaling pathway
  • stimutaling adipogenesis via C/EBP beta-dependent and -independent mechanisms
  • playing a role in the transactivation of C/EBP beta in a hormonal inducers dependent manner
  • its expression in Schwann cells was mediated by AKT1
  • EGR1, EGR2, EGR3, are essential for conversion of the mitogenic signal of epidermal growth factor into a proliferative response
  • specifically required for Schwann cells to initiate and maintain myelination
  • EGR1, EGR2 are novel DNA-binding proteins involved in vertebrate tendon differentiation by regulating type I collagen production
  • EGR2 expressed in restricted stages of lymphocyte development plays a dynamic, but similar role for the development of T, NKT and B cells
  • functions as a SUMO ligase for its coregulators--the Nab proteins--and NAB2 sumoylation negatively modulates EGR2 transcriptional activity
  • important modulator of TNFSF11-induced osteoclast differentiation, providing the link between TNFSF11, EGR2 and ID proteins in osteoclast-lineage cells
  • EGR2 and EGR3 regulate B and T cell function in adaptive immune responses and homeostasis by promoting antigen receptor signaling and controlling inflammation
  • is an essential transcriptional regulator of the T cell anergy program
  • EGR2-neurons are a critical component of the respiratory chemoreflex into adulthood
  • appears to regulate the expression of immunomodulatory molecules
  • EGR2 and EGR3 are the unique regulators for systemic autoimmunity
    • CELLULAR PROCESS nucleotide, transcription, regulation
    PHYSIOLOGICAL PROCESS behavioral , development
    text
  • brain development
  • peripheral nervous system development, mechanosensory behavior
    • PATHWAY
    metabolism
    signaling
    calcineurin signaling pathway
    a component
    INTERACTION
    DNA binding
    RNA
    small molecule metal binding,
  • Zn2+
    • protein
  • binding to the MECP2 promoter and MECP2 binding to the enhancer region in EGR2 intron 1
  • interaction with ADAM19 (functions as a modulator of juxtacrine signaling from axons that activate the AKT pathway and the EGR2 expression)
  • colocalization of EGR2 binding with SOX10, a transcription factor required for Schwann cell specification and other stages of Schwann cell development
  • YY1 emerges as an important activator of peripheral myelination that links neuregulin signaling with EGR2 expression
  • UBE2I is an interaction partner of the transcription factor EGR2, a key regulator of hindbrain development
  • negatively regulates TNFSF11-induced osteoclast differentiation
    • cell & other
    REGULATION
    ASSOCIATED DISORDERS
    corresponding disease(s) CMT1D , CMT4E
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    tumoral     --low  
    in ovarian tumor by hypermethylation in intron 1
    constitutional     --over  
    of EGR1 and EGR2 controls natural killer T lineage differentiation in response to TCR signaling (Pubmed 22306690)
    Susceptibility to Ewing sarcoma
    Variant & Polymorphism SNP
  • rs224278 associated with susceptibility to Ewing sarcoma
    • Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS
  • Egr2-null mice die perinatally of respiratory insufficiency characterized by subnormal respiratory rate and severe apneas