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FLASH GENE
Symbol CENPJ contributors: mct/ - updated : 09-11-2016
HGNC name centromere protein J
HGNC id 17272
Corresponding disease
MCPH6 microcephaly primary 6
SCKL4 Seckel syndrome 4
Location 13q12.12      Physical location : 25.456.413 - 25.497.085
Synonym name
  • LAG-3-associated protein
  • LYST-interacting protein LIP7
  • centrosomal P4.1-associated protein
  • Synonym symbol(s) LAP, CPAP, LIP1, BM032, MGC131581, MGC131582, MGC142222, MGC142224, SASS4
    DNA
    TYPE functioning gene
    STRUCTURE 40.67 kb     17 Exon(s)
    10 Kb 5' upstream gene genomic sequence study
    MAPPING cloned Y linked N status provisional
    RNA
    TRANSCRIPTS type messenger
    identificationnb exonstypebpproduct
    ProteinkDaAAspecific expressionYearPubmed
    17 - 5187 152.9 1338 - 2002 12198240
    EXPRESSION
    Type
       expressed in (based on citations)
    organ(s)
    SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
    Digestiveintestinesmall intestine   
    Lymphoid/Immunelymph node    
    Reproductivefemale systembreastmammary gland  
    tissue
    SystemTissueTissue level 1Tissue level 2LevelPubmedSpeciesStageRna symbol
    Blood / Hematopoieticbone marrow   
    Epithelialbarrier liningneuroepithelium  
    Muscularstriatumskeletal  
    cell lineage
    cell lines breast cancer cell lines
    fluid/secretion
    at STAGE
    physiological period fetal
    Text developing mammary gland
    PROTEIN
    PHYSICAL PROPERTIES
    STRUCTURE
    motifs/domains
  • a leucine zipper, which may form a series of heptad repeats involved in coiled-coil formation, and with five coiled-coil domains
  • glycine repeats
  • microtubule-destabilizing motif that may regulate microtubule dynamics at the centrosome
  • two classical 14-3-3 binding sites, within the C-terminal domain
  • HOMOLOGY
    interspecies homolog to Drosophila Lk6
    homolog to C.elegans R166.5
    Homologene
    FAMILY
  • TCP10 family
  • CATEGORY regulatory , structural protein
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm,cytosolic
    intracellular,cytoplasm,cytoskeleton,microtubule,centrosome
    intracellular,cytoplasm,cytoskeleton,microtubule,mitotic spindle
    intracellular,nucleus
    text
  • localized in the center of microtubules asters and to the spindle poles of mitotic cells
  • also localizes at the midbody ring
  • phosphorylated CENPJ is preferentially located at the procentriole
  • basic FUNCTION
  • centriolar protein that may participate in microtubule nucleation
  • may play an important role in cell division and centrosome function
  • coactivator of STAT5 proteins in the prolactin (and probably other) pathway
  • CENPJ and CP110 play antagonistic roles in determining the extent of tubulin addition during centriole elongation, thereby controlling the length of newly formed centrioles (
  • new regulator of centriole length and its intrinsic tubulin-dimer binding activity is required for procentriole elongation
  • both monomeric and dimeric CENPJ are required for accurate cell division, suggesting that the temporal dynamics of CENPJ homodimerization is tightly regulated during the cell cycle
  • phosphorylated CENPJ is involved in procentriole assembly, possibly for centriole elongation
  • shared function for CENPJ, CEP120, SPICE1 in centriole length control
  • CELLULAR PROCESS cell cycle, division
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism
    signaling
    a component
  • part of the gamma-tubulin complex
  • with CENPJ formed a core structure within the proximal lumen of both parental and nascent centrioles
  • INTERACTION
    DNA
    RNA
    small molecule
    protein
  • binding to the head domain of the 135kDa protein 4.1R isoform (4.1R-135)
  • interacting with STAT5A, STAT5B
  • interaction with YWHAQ, YWHAZ, YWHAG (interaction appears to be phosphorylation and cell cycle dependent, significantly reduced in mitotic cells, suggesting that 14-3-3 binding to CENPJ is regulated during cell cycle progression)
  • interacts with tubulin and controls centriole length
  • substrate of PLK2 whose activity oscillates during the cell cycle
  • interacting with CEP152 (recruits PLK4 and CENPJ to the centrosome to ensure a faithful centrosome duplication process)
  • STIL interacts with the centromere protein CENPJ and rapidly shuttles between the cytoplasm and centrioles
  • CENPJ degradation and function is controlled by the poly(ADP-ribose) polymerase TNKS
  • CEP135 also interacts with another microcephaly protein CENPJ via its N terminal domain
  • CEP120 and SPICE1 cooperate with CENPJ in centriole elongation
  • CEP120 is a cell cycle-regulated protein that directly interacts with CENPJ and is required for centriole duplication
  • CNTROB-CENPJ interaction is critical for the recruitment of CENPJ to procentrioles to promote the elongation of daughter centrioles and for the persistence of CENPJ on preexisting mother centrioles
  • CEP135 physically links to SASS6 near the site of microtubule nucleation and binds to CENPJ for triplet microtubule formation
  • SYCP2 N-reminal region interacts with the centromere during meiosis I, with CENPJ and CENPF
  • cell & other
    REGULATION
    Other cell cycle-regulated phosphorylation orchestrates the dynamics of CENPJ molecular interaction and centrosome splitting to ensure genomic stability in cell division
    ASSOCIATED DISORDERS
    corresponding disease(s) MCPH6 , SCKL4
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    constitutional     --over  
    overexpression in human cells enhances the accumulation of centriolar tubulin, leading to centrioles of strikingly increased length
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS