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Bardet‐Biedl Sydrome (BBS) proteins play a role in Centrosome Amplification
Author(s) -
Teixeira Miguel G,
Zhang Qihong,
Sheffield Val C,
Kooyman David L
Publication year - 2010
Publication title -
the faseb journal
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.709
H-Index - 277
eISSN - 1530-6860
pISSN - 0892-6638
DOI - 10.1096/fasebj.24.1_supplement.507.3
Subject(s) - centrosome , microbiology and biotechnology , biology , bardet–biedl syndrome , phenotype , hek 293 cells , carcinogenesis , microtubule , mutant , cilium , cell culture , cell , genetics , cancer , cell cycle , gene
Many cancers result from mitotic or apoptotic abnormalities. We and others have previously shown that BBS proteins are involved in centrosome organization and regulation. We observed centrosomal clusters in renal epithelial primary cells obtained from BBS mutant mice. Along with the extra centrosomes, we observed a marked increase in the amount of gamma‐tubulin in BBS renal epithelial cells compared to wild type controls. It was unclear if these phenomena were related to the cell culture or a hallmark of BBS mutations. Therefore, we compared BBS mutant and wild type primary renal epithelial cells in culture with kidney sections of the same mice. Although the culture of cells exacerbates the phenotype, we demonstrate a definitive increase in centrosome number within cells in fixed kidneys obtained from BBS mutant mice as compared to wild type controls. We hypothesize that BBS proteins are involved in the regulation or transport of proteins destined for proteosome degradation. Specifically targeting cancer cells with multiple centrosomes presents a viable and novel therapy. Confirming BBS proteins role in centrosome regulation may help to better understand tumorigenesis. This work was supported by the NIH and HHMI.