Centrosomal Protein 110kDa (CEP110)

FAN; CP110; CEP1; Centriolin; Centrosomal Protein 1

SPN	U475
HGNC	1858
MGI	1889576
PubMed	11956314
RGD	1305317
UniProt	Q7Z7A1
Wiki	CEP110

Centriolin is a centrosomal protein required for the centrosome to function as a microtubule organizing center. The gene product is also associated with centrosome maturation. One version of stem cell myeloproliferative disorder is the result of a reciprocal translocation between chromosomes 8 and 9, with the breakpoint associated with fibroblast growth factor receptor 1 and centrosomal protein 1. A chromosomal aberration involving CEP110 may be a cause of stem cell myeloproliferative disorder (MPD). MPD is characterized by myeloid hyperplasia, eosinophilia and T-cell or B-cell lymphoblastic lymphoma. In general it progresses to acute myeloid leukemia. The fusion protein CEP110-FGFR1 is found in the cytoplasm, exhibits constitutive kinase activity and may be responsible for the transforming activity.

Reference

"FGFR1 is fused to the centrosome-associated protein CEP110 in the 8p12 stem cell myeloproliferative disorder with t(8;9)(p12;q33)."
Blood 95:1788-1796(2000) [PubMed] [Europe PMC] [Abstract]

"A novel human protein of the maternal centriole is required for the final stages of cytokinesis and entry into S phase."
J. Cell Biol. 161:535-545(2003) [PubMed] [Europe PMC] [Abstract]

"The full-ORF clone resource of the German cDNA consortium."
BMC Genomics 8:399-399(2007) [PubMed] [Europe PMC] [Abstract]

"DNA sequence and analysis of human chromosome 9."
Nature 429:369-374(2004) [PubMed] [Europe PMC] [Abstract]

"The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]

"Humoral detection of leukaemia-associated antigens in presentation acute myeloid leukaemia."
Biochem. Biophys. Res. Commun. 335:1293-1304(2005) [PubMed] [Europe PMC] [Abstract]

"Characterization of long cDNA clones from human adult spleen. II. The complete sequences of 81 cDNA clones."
DNA Res. 10:49-57(2003) [PubMed] [Europe PMC] [Abstract]

"CEP110 and ninein are located in a specific domain of the centrosome associated with centrosome maturation."
J. Cell Sci. 115:1825-1835(2002) [PubMed] [Europe PMC] [Abstract]

"Centriolin anchoring of exocyst and SNARE complexes at the midbody is required for secretory-vesicle-mediated abscission."
Cell 123:75-87(2005) [PubMed] [Europe PMC] [Abstract]

"Hook2 localizes to the centrosome, binds directly to centriolin/CEP110 and contributes to centrosomal function."
Traffic 8:32-46(2007) [PubMed] [Europe PMC] [Abstract]