centromere protein J; CENP-J; centrosomal P4.1-associated protein; LAG-3-associated protein; LYST-interacting protein 1 (CENPJ, CPAP, LAP, LIP1)

Function

• role in cell division & centrosome function by participation in centriole duplication
• inhibits microtubule nucleation from the centrosome
• phosphorylation at Ser-589 & Ser-595 by PLK2 is required for procentriole formation & centriole elongation
• phosphorylation by PLK2 oscillates during the cell cycle: it increases at G1/S transition & decreases during the exit from mitosis
• phosphorylation at Ser-595 is also mediated by PLK4 but is not a critical step in PLK4 function in procentriole assembly
• part of a ternary complex composed of SASS6, CENPJ & CEP350
• associated with the gamma-tubulin complex
• interacts with the head domain of EPB41
• interacts with LYST
• interacts with CEP152 (via C-terminus)

Structure

belongs to the TCP10 family

Compartment

• cytoplasm, cytoskeleton, centrosome, centriole
• localized within the center of microtubule asters
• during centriole biogenesis, it is concentrated within the proximal lumen of both parental centrioles & procentrioles

Pathology

• defects in CENPJ are the cause of
  • microcephaly primary type 6
  • Seckel syndrome type 4

More general terms

• centrosomal protein
• phosphoprotein

Database

• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=55835
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:55835
• OMIM: https://mirror.omim.org/entry/608393
• OMIM: https://mirror.omim.org/entry/609279
• OMIM: https://mirror.omim.org/entry/613676
• UniProt: http://www.uniprot.org/uniprot/Q9HC77.html