Background: Pericentrin (PCNT) is a large centrosomal protein that binds calmodulin and mediates assembly of the mitotic spindle apparatus. Depletion of PCNT has been shown to cause apoptosis and activation of the G2/M checkpoint. Recently, mutations in the pericentrin gene have been identified as the cause of primordial dwarfism type II (MOPDII) and Seckel syndrome. Additionally, in cells of Seckel syndrome patients, PCNT has been found to be important to the DNA damage response and ATR-dependent checkpoint signaling.