PCM1

PCM1 is a component of centriolar satellites, which are electron dense granules scattered around centrosomes. Inhibition studies show that this protein is essential for the correct localization of several centrosomal proteins, and for anchoring microtubules to the centrosome. Chromosomal aberrations involving this gene are associated with papillary thyroid carcinomas and a variety of hematological malignancies, including atypical chronic myeloid leukemia and T-cell lymphoma.

Full Name

Pericentriolar Material 1

Function

Required for centrosome assembly and function (PubMed:12403812, PubMed:15659651, PubMed:16943179).
Essential for the correct localization of several centrosomal proteins including CEP250, CETN3, PCNT and NEK2 (PubMed:12403812, PubMed:15659651).
Required to anchor microtubules to the centrosome (PubMed:12403812, PubMed:15659651).
Also involved in cilium biogenesis by recruiting the BBSome, a ciliary protein complex involved in cilium biogenesis, to the centriolar satellites (PubMed:20551181, PubMed:24121310, PubMed:27979967).
Recruits the tubulin polyglutamylase complex (TPGC) to centriolar satellites (PubMed:34782749).

Biological Process

Centrosome cycleManual Assertion Based On ExperimentIMP:BHF-UCL
Cilium assemblyManual Assertion Based On ExperimentIMP:UniProtKB
Cytoplasmic microtubule organizationManual Assertion Based On ExperimentIMP:UniProtKB
Interkinetic nuclear migrationISS:BHF-UCL
Intraciliary transport involved in cilium assemblyManual Assertion Based On ExperimentIMP:UniProtKB
Microtubule anchoringISS:BHF-UCL
Microtubule anchoring at centrosomeManual Assertion Based On ExperimentIBA:GO_Central
Negative regulation of neurogenesisISS:BHF-UCL
Neuron migrationIEA:Ensembl
Neuronal stem cell population maintenanceIEA:Ensembl
Non-motile cilium assemblyManual Assertion Based On ExperimentIMP:GO_Central
Positive regulation of intracellular protein transportManual Assertion Based On ExperimentIMP:UniProtKB
Protein localization to centrosomeManual Assertion Based On ExperimentIMP:SYSCILIA_CCNET
Social behaviorIEA:Ensembl

Cellular Location

Cytoplasm, cytoskeleton
Cytoplasm, cytoskeleton, microtubule organizing center, centrosome
Cytoplasmic granule
Cytoplasm, cytoskeleton, microtubule organizing center, centrosome, centriolar satellite
Cytoplasm, cytoskeleton, cilium basal body
Recruitment to the centrosome requires microtubules and dynein. The majority of the protein dissociates from the centrosome during metaphase and subsequently localizes to the cleavage site in telophase. Displaced from centriolar satellites and centrosome in response to cellular stress, such as ultraviolet light (UV) radiation or heat shock, in a process that requires p38 MAP kinase signaling.

Involvement in disease

A chromosomal aberration involving PCM1 is found in papillary thyroid carcinomas (PTCs) (PubMed:10980597). Translocation t(8;10)(p21.3;q11.2) with RET links the protein kinase domain of RET to the major portion of PCM1 (PubMed:10980597).
A chromosomal aberration involving PCM1 is found in a variety of hematological malignancies including atypical chronic myeloid leukemia (atypical CML) and T-cell lymphoma (PubMed:15805263, PubMed:16034466, PubMed:16091753, PubMed:16769584, PubMed:16424865). Translocation t(8;9)(p22;p24) with JAK2 links the protein kinase domain of JAK2 to the major portion of PCM1 (PubMed:15805263, PubMed:16034466, PubMed:16091753, PubMed:16769584, PubMed:16424865).

PTM

Ubiquitinated. Undergoes monoubiquitination catalyzed by the E3 ubiquitin-protein ligase MIB1 in proliferating cells, preventing cilia formation (PubMed:24121310).
Monoubiquitination by MIB1 is inhibited in response to cellular stress, such as ultraviolet light (UV) radiation or heat shock, resulting in cilia formation initiation (PubMed:24121310).
Variant Ser-159 is phosphorylated.
Phosphorylated on multiple serine and threonine residues by DYRK3 during the G2-to-M transition, after the nuclear-envelope breakdown (PubMed:29973724).
Phosphorylation by DYRK3 promotes disassembly of pericentriolar material (PubMed:29973724).
Phosphorylation at Ser-372 mediated by PLK4 is required to maintain the integrity of centriolar satellites (PubMed:30804208).