pericentriolar material 1 protein (PCM-1, hPCM-1, PCM1)
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Function
- required for centrosome assembly & function
- essential for correct localization of several centrosomal proteins including CEP250, CETN3, PCNT & NEK2
- required to anchor microtubules to the centrosome
- self-associates
- interacts with BBS4, BBS8, CETN3, HAP1, NDE1 & NDEL1
Structure
- belongs to the PCM1 family
Compartment
- cytoplasm, centrosome
- localizes to cytoplasmic granules which are enriched around the centrosome
- centrosomal enrichment requires microtubules & dynein
- majority of the protein dissociates from the centrosome during metaphase & subsequently localizes to cleavage site in telophase
- phosphorylated upon DNA damage, probably by ATM or ATR
Alternative splicing
named isoforms=3
Expression
- expressed in blood, bone marrow, breast, lymph node, ovary & thyroid
Pathology
- expression is reduced in breast & ovarian cancer.
- chromosomal translocation t(8;10)(p21.3;q11.2) involving PCM1 with RET is found in thyroid papillary carcinoma the translocation links the protein kinase domain of RET to the major portion of PCM1
- chromosomal aberration involving PCM1 is found in a variety of hematological malignancies including atypical CML & T-cell lymphoma
- chromosomal translocation t(8;9)(p22;p24) involving PCM1 with JAK2 links the protein kinase domain of JAK2 to the major portion of PCM1