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FLASH GENE
Symbol CEP250 contributors: npt/mct/pgu - updated : 16-02-2015
HGNC name centrosomal protein 250kDa
HGNC id 1859
EXPRESSION
Type
   expressed in (based on citations)
organ(s)
SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
Digestiveintestinelarge intestinecolon  
Lymphoid/Immunelymph node    
Nervousbrain    
cell lineage
cell lines
fluid/secretion
at STAGE
cell cycle     cell cycle, interphase
PROTEIN
PHYSICAL PROPERTIES
STRUCTURE
motifs/domains
mono polymer homomer , monomer , dimer
HOMOLOGY
interspecies homolog to murine Cep2
Homologene
FAMILY
CATEGORY DNA associated
SUBCELLULAR LOCALIZATION     intracellular
intracellular,cytoplasm,cytosolic
intracellular,cytoplasm,cytoskeleton,microtubule,centrosome
text
  • in interphase, beta-catenin colocalizes with CROCC between CEP250 puncta at the proximal end of centrioles, and this localization is dependent on CEP250 and CROCC
  • present only at the ends of the centrioles
  • at the onset of mitosis, when centrosomes separate for bipolar spindle formation, dissociates from centrosomes
  • closely co-localized with SCYL1 at the proximal ends of centrioles
  • basic FUNCTION
  • thought to play an important role in centrosome cohesion during interphase of the cell cycle
  • regulation of centriole-centriole cohesion during cell cycle
  • implicated in centrosome cohesion
  • cooperates with CEP68 and CROCC in centrosome cohesion
  • CEP250 or CROCC knockdown reduced primary cilium formation
  • interphase centrosomes are physically tethered by a proteinaceous linker composed of CEP250 and the filamentous protein rootletin (CROCC)
  • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism
    signaling
    a component
  • forms a complex in vitro with both NEK2 kinase and the
  • PPP1CC catalytic subunit of protein phosphatase 1 (PP1)
  • centrosome cohesion is maintained by the newly identified complex of CEP250-CNTLN-CEP68
  • INTERACTION
    DNA
    RNA
    small molecule
    protein
  • interacts with CROCC
  • interaction between CEP135 and CEP250, two core centriolar proteins
  • AXIN2, a negative regulator of beta-catenin, localizes at the centrosomes by binding to the centriole-associated component CEP250
  • SIK2 plays a key role in the initiation of mitosis, and regulates the localization of the centrosome linker protein, CEP250, through S2392 phosphorylation
  • interaction with PLK1 (PLK1 is required for both CEP250 displacement and for KIF11 localization on the centrosome)
  • interaction partners of CEP135, the 50 kDa subunit of the dynactin complex (DCTN2) and CEP250, have their binding sites in the C-terminal domain of CEP135
  • recruitment of NEK2 to the proximal ends of centrioles is dependent on one of its substrates, the centrosome cohesion protein CEP250
  • LRRC45 interacts with both CEP250 and CROCC and is phosphorylated by NEK2 at S661 during mitosis
  • CNTLN localizes to the proximal ends of the centrioles and directly interacts with both CEP250 and CEP68
  • phosphorylation of CEP250 perturbs interaction with the core centriolar protein, CEP135, and interaction of endogenous CEP250 and CEP135 proteins is specifically lost in mitosis
  • centriolar loading of SCYL1 stimulated by EGF/AKT could displace CEP250, resulting in centrosome splitting
  • centriolar protein CNTLN functions as a molecular link between CEP250 and CEP68 to maintain centrosome cohesion
  • cell & other
    REGULATION
    Other dissociation from mitotic centrosomes is regulated by localized phosphorylation rather than generalized proteolysis
    ASSOCIATED DISORDERS
    ANIMAL & CELL MODELS