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FLASH GENE
Symbol CETN2 contributors: mct/pgu - updated : 19-08-2015
HGNC name centrin, EF-hand protein, 2
HGNC id 1867
PROTEIN
PHYSICAL PROPERTIES
STRUCTURE
motifs/domains
  • N-terminal domain having a closed structure, binds calcium with a very low affinity, and plays a role in the protein self-assembly
    • mono polymer monomer
    HOMOLOGY
    interspecies homolog to yeast cdc31
    homolog to murine Cetn2
    Homologene
    FAMILY
  • EF-hand superfamily of calcium-binding proteins
    • CATEGORY structural protein
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm,cytosolic
    intracellular,cytoplasm,cytoskeleton,microtubule,centrosome
    intracellular,nucleus,chromatin/chromosome,centromere
    intracellular,nucleus,chromatin/chromosome,kinetochore
    intracellular,nuclear envelope,pore
    text
  • distal lumen of centrioles, centrosome of interphase and mitotic cells
  • localizes to the vertebrate nuclear pore
  • associated with centrosome-related structures
    • basic FUNCTION
  • involved in structure and function of microtubule organizing center, duplication and segregation of centrosome
  • stimulating nucleotide excision repair by interacting with xeroderma pigmentosum group C protein
  • plays a functional role in multiple nuclear export pathways
  • has the ability to bind to any of the approximately 25 repeats of human SFI1 (hSfi1) with more or less affinity
  • can organize a subset of centriolar proteins independently of cartwheels
  • its phosphorylation potentially stimulates the canonical centriole assembly pathway
    • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism
    signaling
    a component
  • core component of the centrosome of higher eukaryotes
  • forms a strong heterotrimeric complex with RAD23B and XPC
    • INTERACTION
    DNA
    RNA
    small molecule metal binding,
  • Ca2+
    • protein
  • XPC (stabilizes XPC in cooperation with HR23B), and localization of centrin-2 within the nucleus depends on its ability to bind to the XPC protein
  • interacting with SFI1 (role of centrin-SFI1 interactions in the dynamic structure of centrosome-associated contractile fibers)
  • associate biochemically with nucleoporins
  • assemble into protein complexes with the visual heterotrimeric G-protein transducin, interaction mediated by binding to the betagamma-dimer of the heterotrimeric G-protein
  • substrate of SUMOylation, and it is preferentially modified by SUMO2/3
  • through activation of a centrosomal pool of CDK2, CDC25B stabilises the local pool of Monopolar spindle 1 (Mps1) which in turn regulates the level of CETN2 at the centrosome
  • CETN2 regulates primary ciliogenesis through controlling CCP110 levels
    • cell & other
    REGULATION
    Other regulated by TTK (TTK-dependent phosphorylation of CETN2 stimulates the canonical centriole assembly pathway)
    ASSOCIATED DISORDERS
    corresponding disease(s)
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    constitutional     --over  
    causes MPS1-dependent centriole overproduction that requires each of the three TTK phosphorylation sites within CETN2 and is greatly exacerbated by mimicking phosphorylation at any of these sites
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS