Pcnt-KO Mouse

Pcnt, also known as pericentrin, is a key pericentriolar material (PCM) protein. The centrosome, consisting of centrioles and PCM, is crucial for mitotic spindle assembly, and Pcnt plays a role in microtubule nucleation and organization during interphase and mitosis [4].

Deletion of Pcnt had little effect on interphase centrosomes, but in mitotic cells, centrioles prematurely separated and frequently amplified, indicating that Pcnt limits centrioles within the spindle poles during mitosis [1]. Specific cleavage of Pcnt is necessary for centriole separation during mitotic exit, and its abnormal cleavage affects centriole separation and conversion [1]. In hepatocellular carcinoma (HCC), knockdown of Pcnt expression inhibited cell viability, migration, and invasion, suggesting its role in promoting tumor progression [2]. Biallelic loss-of-function mutations in Pcnt cause microcephalic osteodysplastic primordial dwarfism type II (MOPDII), characterized by growth retardation, microcephaly, etc. [3]. Missense mutations in Pcnt were also found in patients with intracranial aneurysms or oral and maxillofacial osteodysplasia [4,5].

In conclusion, Pcnt is essential for the proper functioning of centrosomes during mitosis, especially in centriole behavior. Its abnormal function is associated with various diseases such as cancer, MOPDII, and intracranial aneurysms. The study of Pcnt knockout or mutant models helps to understand its biological functions and the mechanisms underlying related diseases.