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FLASH GENE
Symbol TOPORS contributors: mct - updated : 15-03-2011
HGNC name topoisomerase I binding, arginine/serine-rich
HGNC id 21653
PROTEIN
PHYSICAL PROPERTIES
STRUCTURE
motifs/domains
  • N-terminal RING-type zinc-finger domain (amino acids 103
  • 141)
  • five stretches enriched in proline, glutamic acid, serine, and threonine (PEST) residues
  • a bipartite nuclear localization signal
  • a region rich in Arg-Ser
  • coiled-coil structure and leucine zipper
  • two putative SUMO-1 conjugation sites
  • HOMOLOGY
    interspecies homolog to murine Topors (87.4pc)
    Homologene
    FAMILY
  • ring finger protein family
  • CATEGORY tumor suppressor
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm,cytoskeleton,microtubule,centrosome
    intracellular,nucleus,nucleoplasm,nuclear bodies,nuclear speckles
    text
  • cilia-centrosomal protein
  • basic FUNCTION
  • trans-acting transcriptional regulator of E-cadherin and talin gene in the alveolar epithelial cell during lung development, differentiation, tumorigenesis
  • E3 ubiquitin ligase with specific E2 enzymes, and it can ubiquitinate TP53
  • can function as both an ubiquitin and SUMO-1 E3 ligase for TP53
  • enhancing the formation of high molecular weight SUMO-1 conjugates of TOP1
  • potential binary switch function for TOPORS in protein ubiquitination versus sumoylation
  • ubiquitin and small ubiquitin-like modifier ubiquitin-protein isopeptide ligase (SUMO E3) ligase
  • involved in normal mitotic progression, since its depletion delays mitotic entry and affects mitotic progression
  • has both ubiquitin and SUMO E3 ligase activity
  • unique role in the maintenance of genomic stability and pericentric heterochromatin, as well as in cellular sensitivity to histone deacetylase inhibitors
  • may play a key role in regulating primary cilia-dependent photoreceptor development and function
  • promotes proteasomal degradation of NKX3.1 through direct interaction, predisposing cells to oncogenic transformation by providing an irreversible growth advantage as the first steps in prostate carcinogenesis
  • CELLULAR PROCESS nucleotide, transcription, regulation
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism
    signaling
    a component
  • component of the photoreceptor sensory cilium, which is a modified primary cilium involved with polarized trafficking of proteins
  • INTERACTION
    DNA binding
    RNA
    small molecule metal binding,
  • ions Zn2+
  • protein
  • potentially binding to the palindromic sequence in the 5'UTR of E-cadherin and in two intervening regions of TALIN
  • negative regulator of NKX3.1 (implicate TOPORS in prostate cancer progression)
  • binding partner of p53 and TOP1
  • PLK1 target (PLK1 phosphorylates TOPORS on Ser(718))
  • cell & other
    REGULATION
    Other PLK1 phosphorylation is involved in its degradation
    ASSOCIATED DISORDERS
    corresponding disease(s) RP31
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    tumoral     --over  
    leads to NKX3.1 proteasomal degradation in prostate cancer cells
    tumoral        
    lung carcinoma
    constitutional somatic mutation insertion    
    causing autosomal dominant retinitis pigmentosa
    constitutional somatic mutation deletion    
    causing autosomal dominant retinitis pigmentosa
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS
    Somatic-gene-therapy rescue has been achieved in several animal models of retinal degeneration, including the rds mouse, and may also prove to be a successful approach for this type of adRP