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Publication : A novel protein LZTFL1 regulates ciliary trafficking of the BBSome and Smoothened.

First Author  Seo S Year  2011
Journal  PLoS Genet Volume  7
Issue  11 Pages  e1002358
PubMed ID  22072986 Mgi Jnum  J:177990
Mgi Id  MGI:5296985 Doi  10.1371/journal.pgen.1002358
Citation  Seo S, et al. (2011) A Novel Protein LZTFL1 Regulates Ciliary Trafficking of the BBSome and Smoothened. PLoS Genet 7(11):e1002358
abstractText  Many signaling proteins including G protein-coupled receptors localize to primary cilia, regulating cellular processes including differentiation, proliferation, organogenesis, and tumorigenesis. Bardet-Biedl Syndrome (BBS) proteins are involved in maintaining ciliary function by mediating protein trafficking to the cilia. However, the mechanisms governing ciliary trafficking by BBS proteins are not well understood. Here, we show that a novel protein, Leucine-zipper transcription factor-like 1 (LZTFL1), interacts with a BBS protein complex known as the BBSome and regulates ciliary trafficking of this complex. We also show that all BBSome subunits and BBS3 (also known as ARL6) are required for BBSome ciliary entry and that reduction of LZTFL1 restores BBSome trafficking to cilia in BBS3 and BBS5 depleted cells. Finally, we found that BBS proteins and LZTFL1 regulate ciliary trafficking of hedgehog signal transducer, Smoothened. Our findings suggest that LZTFL1 is an important regulator of BBSome ciliary trafficking and hedgehog signaling.
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