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FLASH GENE
Symbol PCNT contributors: mct/pgu - updated : 24-06-2011
HGNC name pericentrin
HGNC id 16068
EXPRESSION
Type ubiquitous
   expressed in (based on citations)
organ(s)
SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
Endocrineneuroendocrinepituitary  highly
 pancreasislet of Langerhans  highly Homo sapiens
Lymphoid/Immunelymph node   highly
Reproductivemale systemtestis   
 male systemprostate   
Respiratoryrespiratory tractlarynx  highly
tissue
SystemTissueTissue level 1Tissue level 2LevelPubmedSpeciesStageRna symbol
Blood / Hematopoieticbone marrow   
Muscularstriatumskeletal highly Homo sapiens
cells
SystemCellPubmedSpeciesStageRna symbol
Endocrineislet cell (alpha,beta...) Homo sapiens
cell lineage
cell lines
fluid/secretion
at STAGE
PROTEIN
PHYSICAL PROPERTIES
STRUCTURE
motifs/domains
  • two large N-terminal coiled coil domains bounded by non coiled regions
  • large N-terminal coiled-coil domain of approximately 1,500 residues
  • a region with AAs 8-42 contains a tripartite nuclear localization signal (NLS) consisting of three clusters of basic amino acids
  • five novel nuclear export signals (NESs) with diverse export activities
  • NESs and NLS are essential for its subcellular localization and nucleocytoplasmic trafficking during the cell cycle
  • a smaller C-terminal coiled-coil domain of approximately 500 AAs, the PACT domain that targets it to the centrosome
  • HOMOLOGY
    interspecies homolog to murine pericentrin
    homolog to yeast Spc110
    Homologene
    FAMILY
    CATEGORY structural protein
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm,cytosolic
    intracellular,cytoplasm,cytoskeleton,microtubule,centrosome
    text
  • component of the filamentous matrix of the centrosome, of the pericentriolar material
  • colocalizes with IFT proteins to the base of primary and motile cilia
  • giant coiled-coil protein localized in the pericentriolar materials as an integral component, and serves as a multifunctional scaffold by anchoring numerous proteins and protein complexes
  • in skeletal muscle, showed a perinuclear distribution
  • basic FUNCTION
  • microtubule organization during cell division
  • required for assembly of the mitotic spindle apparatus
  • mediates the nucleation of microtubules by anchoring the gamma-tubulin ring complex, which initiates the assembly of the mitotic spindle apparatus
  • possible role of pericentrin in the development of cerebral vessels
  • MCPH1 and PCNT regulate mitotic entry
  • via centrosome-associated CHEK1
  • anchors NEK2 and suppresses its kinase activity at the centrosomes, and thus, is involved in the mechanism to prevent premature centrosome splitting during interphase
  • may play a pivotal role in preventing premature centrosome splitting during interphase by inhibiting Nek2A kinase activity at the centrosome
  • may regulate the intracellular distribution and secretion of insulin
  • CELLULAR PROCESS cell organization/biogenesis
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism
    signaling
    a component
  • integral constituent of the filamentous matrix of the centrosome
  • complexing with IFT20, PKD2 (complex required for assembly of primary cilia and possibly motile cilia and flagella)
  • INTERACTION
    DNA
    RNA
    small molecule
    protein
  • PCM1
  • cleavage target of MMP14
  • UTP6 and kendrin (PCNT) provide sites for microtubule nucleation in the mammalian centrosome by anchoring TUBG1
  • DISC1–kendrin (PCNT) interaction is indispensable for the centrosomal localization of DISC1
  • associates with NEK2, which exhibits considerably low activity
  • PLK1-mediated phosphorylation of pericentriolar matrix initiates centrosome maturation by organizing the spindle pole-specific PCM lattice
  • cell & other
    REGULATION
    Phosphorylated by specifically phosphorylated by PLK1 during mitosis
    ASSOCIATED DISORDERS
    corresponding disease(s) SCKL3 , MOPD2
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    tumoral     --over  
    in carcinoma cell lines
    constitutional        
    depletion of PCNT induces premature centrosome splitting in interphase
    constitutional       loss of function
    pericentrin deficiency does not impair proximal insulin action in adipocytes
    constitutional     --over  
    in beta-cells resulted in aggregation of insulin-containing secretory vesicles with cytoplasmic, but not centrosomal, pericentriolar material and an increase in total levels of intracellular insulin
    constitutional        
    lack of MCPH1 or PCNT results in a loss of CHEK1 from centrosomes with subsequently deregulated activation of centrosomal cyclin B-CDK1
    Susceptibility to bipolar affective disorder
    Variant & Polymorphism
    Candidate gene for dyslexia
    Marker
    Therapy target
    SystemTypeDisorderPubmed
    diabete  
    target for modulating regulated protein secretion in disorders such as diabetes
    ANIMAL & CELL MODELS