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FLASH GENE
Symbol PCM1 contributors: mct/pgu - updated : 04-01-2017
HGNC name pericentriolar material 1
HGNC id 8727
PROTEIN
PHYSICAL PROPERTIES
STRUCTURE
motifs/domains
  • N-terminal domain of PCM1 can restore ciliogenesis and satellite localization of certain proteins, but not others, pinpointing unique roles for PCM1 and a group of satellite proteins in cilium assembly
  • HOMOLOGY
    interspecies ortholog to murine Pcm1
    Homologene
    FAMILY
  • PCM1 family
  • CATEGORY antigen
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm,cytosolic
    intracellular,cytoplasm,cytoskeleton,microtubule,centrosome
    intracellular,nuclear envelope
    text
  • centrosomal localization of PCM1 in glia but not in neurons
  • co-localizes with GRK5, TUBG1, and CETN1 in centrosomes
  • basic FUNCTION
  • involved in recruiting proteins necessary for centrosome replication
  • participating in the biogenesis, function and maintenance of both centrosomes and cilia
  • plays a role in the recruitment of centrosomal proteins including centrin, pericentrin and ninein, and required for the organization of the cytoplasmic microtubule network
  • involved in the regulation of a membrane-trafficking pathway mediated by RAB8A, which may be coordinated with the assembly of the ciliary axoneme
  • microtubule assembly is PCM1 dependent, and the presence of PCM1 at the centrosome contributes to its role as the main microtubule-organizing centre
  • HOOK3 interacts with PCM1 to regulate pericentriolar material assembly and the timing of neurogenesis
  • implicated in centrosomal protein trafficking
  • promotes ciliogenesis by tethering a key E3 ligase to satellites and restricting it from centrioles
  • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism
    signaling
    a component
  • binding to centrosome in a dynamic cell cycle-dependent manner
  • complexing with BBS4 and colocalizing to the centriolar satellites of centrosomes
  • INTERACTION
    DNA
    RNA
    small molecule
    protein
  • interacting with BBS4
  • interacting with PIBF1 (both PIBF1 and PCM1 are required for ciliogenesis and are involved in the ciliary targeting of RAB8, a small GTPase shown to collaborate with BBS protein complex to promote ciliogenesis)
  • interaction between HOOK3 and PCM1 required by trafficking of pericentriolar satellites
  • interact directly with the disrupted-in-schizophrenia 1 (DISC1) protein, BBS4, and Huntingtin-associated protein 1, and is important in neuronal cell growth
  • HTT regulates ciliogenesis by interacting through HAP1 with pericentriolar material 1 protein (PCM1)
  • HOOK2 interacts with and stabilizes pericentriolar material protein 1 (PCM1), which was reported to be essential for the recruitment of RAB8A, a GTPase that is believed to be crucial for membrane transport to the primary cilium
  • CEP72 is a PCM1-interacting protein required for recruitment of the ciliopathy-associated protein CEP290 to centriolar satellites
  • CEP131 is recruited to centriolar satellites by PCM1, and localised to the centriolar core region by both pericentrin and CEP290
  • PIBF1 physically interacts with PCM1 at centriolar satellites, and this interaction is essential for centrosomal accumulation of the centriolar satellites and eventually for primary cilia formation
  • centrin interactor POC5 leads to the assembly of linear, centrin-dependent structures that recruit other centrosomal proteins such as PCM1 and NEDD1
  • MIB1 is a new component of centriolar satellites, which interacts with and ubiquitylates CEP131 and PCM1 and suppresses primary cilium formation
  • CCDC13 is a newly identified centriolar satellite protein that interacts with PCM1, CEP290 and pericentrin and prevents the accumulation of DNA damage during mitotic transit
  • SDCCAG8 interacts and cotraffics with pericentriolar material 1 (PCM1), a centriolar satellite protein crucial for targeting proteins to the centrosome
  • WRAP73 interacts with the satellite proteins SSX2IP and PCM1 as well as the centriolar proximal end component CEP135
  • PLK4 interacts with the satellite component PCM1, and its kinase activity is required for phosphorylation of the conserved S372
  • PCM1 is essential for tethering the E3 ligase, Mindbomb1 (MIB1), to satellites
  • cell & other
    REGULATION
    ASSOCIATED DISORDERS
    corresponding disease(s)
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    tumoral   translocation    
    t(8;9)(p22;p24), in in atypical chronic myeloid leukaemia with a PCM1-JAK2 fusion
    tumoral fusion      
    fusion with RET in papillary thyroid carcinoma
    tumoral   LOH    
    in hepatocellular carcinoma, colorectal and non-small-cell lung cancers
    tumoral     --low  
    in primary ovarian carcinoma compared with controls (ovarian tissues and cysts)
    Susceptibility to schizophrenia
    Variant & Polymorphism SNP , other
  • rs37042, a threonine to isoleucine missense mutation is strongly associated with schizophrenia
  • Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS