pericentrin

Pericentrin is a large, structural protein of the centrosome, localized to the pericentriolar material (PCM). It is encoded by the PCNT gene in humans and is also known by the alternative name kendrin. As a dominant scaffolding component of the PCM, pericentrin provides a framework that recruits and organizes other centrosomal proteins involved in microtubule nucleation and spindle assembly.

Functionally, pericentrin anchors the γ-tubulin ring complex (γ-TuRC) and interfaces with other PCM proteins such as CEP215/CDK5RAP2, AKAP450, and ninein. Through these interactions, it facilitates microtubule nucleation and stabilizes spindle poles during mitosis. Pericentrin contributes to centrosome maturation, duplication, and the proper orientation of cell division. Its activity is regulated by phosphorylation by kinases including CDKs and PLK1, linking it to cell cycle progression and spindle dynamics.

Localization studies show pericentrin concentrates at the PCM throughout the cell cycle, with coordinated remodeling during centrosome maturation in mitosis. Experimental depletion or dysfunction of pericentrin disrupts PCM organization, impairs microtubule nucleation, and can lead to spindle abnormalities.

Clinical relevance is highlighted by mutations in PCNT, which cause microcephalic osteodysplastic primordial dwarfism type II (MOPDII) and related growth disorders. Affected individuals typically exhibit microcephaly, short stature, and skeletal anomalies. Research on PCNT variants contributes to understanding centrosome biology, spindle assembly, and the basis of certain congenital dwarfism conditions.

Pericentrin is conserved across metazoans and remains a widely used marker of the PCM in cell biology studies, reflecting its central role in organizing the microtubule cytoskeleton at the centrosome.